Terminal2 · Diagnosis Card #59

Scleroderma (Systemic Sclerosis)

An evidence-based clinical reference for clinicians, families, and patients navigating systemic sclerosis at end of life — pulmonary fibrosis and pulmonary hypertension occurring simultaneously, GI dysmotility progressing to intestinal failure, scleroderma renal crisis as a reversible emergency, digital ulcers from Raynaud's vasculopathy, calcinosis, multi-mechanism pain, and the hidden disability of a body that has been turning to stone from the inside out.

S01 Everyone

What Is It

Definition, mechanism, and the clinical reality of systemic sclerosis at end of life. What the hospice team needs to understand on day one.

US Prevalence
~100–300K
Systemic sclerosis affects approximately 100,000–300,000 Americans. Annual incidence ~20 per million per year. SSc is rare and systematically underdiagnosed.[1]
Female:Male Ratio
4–9 : 1
One of the highest female-to-male ratios of any autoimmune disease. SSc is primarily a disease of middle-aged women diagnosed at age 45–55.[2]
SSc-ILD 5-Year Survival
~65–70%
SSc with interstitial lung disease — shorter than many cancers. SSc-PAH 3-year survival is approximately 50–60%.[7]
Subtypes
dcSSc / lcSSc
Diffuse cutaneous SSc (dcSSc) — rapid skin progression, early organ involvement, shorter survival. Limited cutaneous SSc (lcSSc/CREST) — slower progression, PAH risk increases with disease duration.[4]

Systemic sclerosis (scleroderma) is a chronic, progressive, multi-system autoimmune disease characterized by three simultaneous pathological processes: fibrosis, vasculopathy, and immune dysregulation. The fibrosis stiffens and thickens the skin — the hallmark that gives the disease its name — and then works its way into the lungs (interstitial lung disease), the heart (myocardial fibrosis), the gastrointestinal tract (dysmotility from esophagus to rectum), and the kidneys (scleroderma renal crisis). The vasculopathy produces Raynaud's phenomenon as the earliest symptom and progresses to digital ulcers, digital gangrene, and pulmonary arterial hypertension. The immune dysregulation drives the production of disease-specific autoantibodies (anti-Scl-70, anti-centromere, anti-RNA polymerase III) that predict the pattern of organ involvement with striking accuracy.[1]

The typical patient arriving at hospice enrollment is a woman in her 50s or 60s who has been managing SSc for 10–20 years. She has watched her hands change — the skin tightening over the fingers (sclerodactyly), the fingertips developing painful ulcers that refuse to heal, the joints contracting. Her face has become less mobile — the reduced oral aperture (microstomia) that restricts mouth opening, the thinned lips, the reduced facial expression from skin tightening. She has stopped eating much because eating produces reflux, bloating, and pain from the GI dysmotility that affects every level of the gastrointestinal tract. She is short of breath because the lungs are fibrosed, or because pulmonary hypertension has developed, or both. The hospice clinician who walks in must understand that the exterior — the tight skin, the contracted hands, the changed face — is the most visible part of a disease that has been quietly destroying internal organs for years.[2]

SSc disproportionately affects Black women, who develop SSc at younger ages, have more severe internal organ involvement at diagnosis, have higher rates of pulmonary hypertension and interstitial lung disease, and have shorter survival — a disparity that is multifactorial from both biological factors (specific antibody patterns are more common in Black women with SSc) and from healthcare access disparities that delay diagnosis and specialty care. The SSc Registries (EUSTAR in Europe, GENISOS in the US) document these disparities. The hospice clinician must be aware that the Black woman with SSc in her care may have had a more aggressive disease course with less access to specialty rheumatology care than her white counterpart.[56]

🧭 Clinical framing

SSc is the disease that destroys multiple organ systems simultaneously through the same fibrotic and vasculopathic mechanisms — but each organ requires its own specific management approach. The lungs need opioids for dyspnea and continued oxygen. The kidneys need blood pressure monitoring and captopril readiness for scleroderma renal crisis. The GI tract needs prokinetics and PPI optimization. The digital ulcers need vasodilators and pre-procedure analgesia. The pain is multi-mechanism — musculoskeletal, neuropathic, ischemic, visceral — and no single analgesic class addresses it all. The hospice clinician who treats SSc as a single-organ disease will miss the other organs that are simultaneously failing.

From the Field
Waldo Rios, NP
Hospice NP · 12+ Years
"Before you walk into the room, know three things: what antibody she has, whether she's on a vasodilator, and where the captopril is. The antibody tells you her renal crisis risk. The vasodilator tells you about her digital ischemia. The captopril — if she's anti-RNAP III positive, that drug better be in the house and everybody better know where it is. This is the disease where the blood pressure check at every visit isn't routine — it's a screening act for a reversible emergency."
— Waldo, NP · Terminal2
S02Clinician

How It's Diagnosed

SSc classification criteria, autoantibody profiling, organ staging, and what to extract from prior records at hospice enrollment.

2013 ACR/EULAR Classification Criteria
Guideline

SSc classification with score ≥9 from:[1]

  • Skin thickening proximal to MCPs: 9 points (sufficient alone for classification)
  • Sclerodactyly (distal to MCP, proximal to PIP): 4 points
  • Puffy fingers: 2 points
  • Fingertip lesions: Digital ulcers (2 pts) or pitting scars (3 pts)
  • Telangiectasias: 2 points
  • Abnormal nailfold capillaries: 2 points
  • PAH and/or ILD: 2 points
  • Raynaud's phenomenon: 3 points
  • SSc-related antibodies (ACA, anti-Scl-70, anti-RNAP III): 3 points

At hospice enrollment the diagnosis is established — document subtype (dcSSc vs lcSSc), antibody profile, year of diagnosis, and disease duration.

Autoantibody Profile & Prognosis
Review

The antibody is the single most important prognostic predictor:[52]

  • Anti-Scl-70 (anti-topoisomerase I): 20–30% of SSc; associated with dcSSc, ILD risk (70% develop ILD), moderate PAH risk, lower SRC risk
  • Anti-centromere (ACA): 20–30%; associated with lcSSc (CREST), lower ILD risk, high PAH risk (15–30% over 15 years), very low SRC risk
  • Anti-RNA polymerase III (anti-RNAP III): 10–20%; associated with dcSSc, rapid skin progression, HIGHEST SRC risk (25–40%), lower ILD risk

Document the antibody at enrollment — it determines SRC risk and surveillance protocol. The anti-RNAP III patient must be monitored for SRC at every visit.

Organ-Specific Assessment at Enrollment
  • Pulmonary: Most recent PFTs (FVC % predicted — below 70% significant, below 50% severe); HRCT pattern (NSIP vs UIP); most recent echocardiogram (RVSP — above 40 mmHg suggests PAH, above 60 confirms severe PAH); RHC results if available (mPAP ≥20 mmHg = PH; mPAP ≥25 + PCWP ≤15 = PAH)[7]
  • Renal: Baseline creatinine and trend; blood pressure baseline and trend; antibody-specific SRC risk (anti-RNAP III: HIGH); prior SRC episodes; current ACE inhibitor status[25]
  • GI: Esophageal dysmotility assessment; gastroparesis severity; SIBO history and treatment; nutritional status (albumin, BMI, weight trend); fecal incontinence assessment; TPN status or trajectory[39]
  • Cardiac: Echocardiographic EF; pericardial effusion; conduction abnormalities on ECG; BNP/NT-proBNP trend
Functional & Skin Assessment
  • Modified Rodnan Skin Score (mRSS): Palpation of 17 body sites (0–3 at each); total 0–51; higher scores = more extensive skin involvement; in dcSSc, the mRSS typically peaks at 3–5 years then may plateau or partially improve even as internal organs continue to worsen[4]
  • Oral aperture: Measure in centimeters — normal ≥40 mm; below 30 mm is significant microstomia; below 20–25 mm has implications for dental care, nutrition (small bolus requirements), and emergency airway management
  • Hand function: Document grip strength, finger contractures, digital ulcer locations and staging, calcinosis sites
  • Performance status: ECOG/PPS trend over last 3–6 months; functional decline below PPS 40% supports hospice eligibility
  • Digital vascular assessment: Active Raynaud's frequency and severity; digital ulcer inventory; history of digital amputations; current vasodilator regimen

💡 For families

Your loved one has been diagnosed with systemic sclerosis for years — the diagnostic workup is complete. At hospice enrollment, the clinical team reviews the medical history to understand which organs are affected and how far the disease has progressed. The most important things we look for are: which antibody type your person has (this tells us about kidney risk), how the lungs are functioning, and how the digestive system is working. This information helps us build the most effective comfort plan.

S03Everyone

Causes & Risk Factors

SSc pathogenesis — fibrosis, vasculopathy, and immune dysregulation — and the clinical relevance for end-of-life management.

The Fibrosis Mechanism
  • TGF-β (transforming growth factor beta) is the master regulator of fibrosis in SSc — overproduced by activated fibroblasts and myofibroblasts, driving collagen deposition that stiffens skin, lungs, heart, and gut[15]
  • Fibrosis is irreversible at end stage — the collagen deposited does not regress with immunosuppressant therapy; disease-modifying therapies (mycophenolate, cyclophosphamide, tocilizumab, nintedanib) slow the rate of fibrosis accumulation but cannot reverse established fibrosis
  • Clinical implication for hospice: The structural changes of end-stage SSc are largely fixed — the tightened skin, the restricted lung, the fibrotic gut. Management focus must be on comfort for symptoms produced by those fixed structural changes rather than further attempts to slow completed fibrosis
The Vasculopathy & Raynaud's
  • SSc vasculopathy produces fixed narrowing and obliteration of digital arteries that persists even between Raynaud's attacks; the combination of fixed structural narrowing plus episodic vasospasm produces ischemia that causes digital tip ulcers[31]
  • Raynaud's phenomenon: Episodic vasospasm triggered by cold or stress producing triphasic color change (pallor → cyanosis → erythema); more severe in SSc than primary Raynaud's because of reduced microvascular reserve
  • Endothelin-1 is overproduced in SSc — the most potent endogenous vasoconstrictor, driving both digital ischemia and pulmonary arterial hypertension; this is the target of bosentan and macitentan
  • Digital ulcers at the fingertips are simultaneously the most painful and most refractory wounds in rheumatological disease — they occur at the most densely innervated surface of the body
Immune Dysregulation & Autoantibodies
  • SSc autoantibodies are not just diagnostic markers — they predict the pattern of organ involvement with striking specificity: anti-Scl-70 predicts ILD; anti-centromere predicts PAH; anti-RNAP III predicts scleroderma renal crisis[52]
  • B-cell and T-cell activation drive the inflammatory phase of SSc that precedes and promotes fibrosis; this is the target of rituximab and tocilizumab
  • The immune-fibrotic transition: Early SSc is immune-driven (inflammatory, potentially modifiable); late SSc is fibrosis-driven (structural, largely irreversible). Hospice patients are typically in the fibrosis-driven phase
Risk Factors & Disparities
  • Environmental triggers: Silica dust exposure (RR 2–5×); organic solvents; vinyl chloride; bleomycin and other drugs can induce SSc-like syndromes[2]
  • Genetic susceptibility: HLA associations (HLA-DRB1 alleles); first-degree relatives have 10–16× increased SSc risk; concordance in twins is low (~5%), confirming environmental-genetic interaction
  • Racial disparities: Black women develop SSc at younger ages with more severe organ involvement, higher rates of ILD and PAH, and shorter survival — from both biological factors and healthcare access disparities[56]
  • Sex disparity: 4–9:1 female predominance, likely from X-chromosome gene dosage effects, fetal microchimerism, and estrogen-related immune modulation

❤️ For families: "Why did this happen?"

Systemic sclerosis is not caused by anything your loved one did or didn't do. It results from a complex interaction of genetic susceptibility and environmental triggers that science is still working to understand. No dietary choice, no stress event, no lifestyle decision caused this disease. In some cases, occupational exposure to silica or certain chemicals may have contributed — but even then, the disease requires an underlying immune predisposition. What matters now is not causation but care.

⚕ Clinician note: Calcinosis pathogenesis

Calcinosis cutis in SSc involves deposition of hydroxyapatite calcium crystite in subcutaneous tissue, often at pressure points (fingertips, elbows, knees). The calcium deposits can extrude through the skin, creating open wounds that are painful and infection-prone. The pathogenesis involves tissue damage → inflammation → dystrophic calcification in a cycle that is self-perpetuating. No treatment reliably resolves established calcinosis — management is wound-based and pain-focused. Colchicine, warfarin, diltiazem, and minocycline have been tried with inconsistent results.[36]

S04ClinicianEveryone

Treatments & Procedures

Multi-system SSc management at hospice enrollment — pulmonary, renal, GI, vascular, and pain. Understanding prior therapy and what continues at comfort focus.

SSc at hospice enrollment requires simultaneous management of multiple organ systems, each with its own medication regimen and specific contraindications. The patient may arrive on 10–15 medications addressing lungs, heart, GI tract, kidneys, blood vessels, and pain — each prescribed by a different specialist. The hospice clinician's first task is to inventory every medication, understand its target organ, and assess its ongoing comfort benefit versus burden.[7]

SSc-ILD Management (Card #43 Framework)
RCT
  • Nintedanib (Ofev) 150 mg BID: FDA-approved for SSc-ILD (SENSCIS trial, Distler 2019 NEJM) — reduces rate of FVC decline. At hospice enrollment, assess comfort-benefit: does it provide symptom benefit (slowing dyspnea progression) or primarily adding nausea/diarrhea burden?[7]
  • Mycophenolate mofetil (CellCept): SLS-II trial demonstrated comparable efficacy to cyclophosphamide for SSc-ILD with better tolerability. At end stage, reassess benefit[8]
  • Opioids for dyspnea: Low-dose morphine 2.5–5 mg PO/SQ q4h as in IPF — the same evidence base applies
  • Oxygen therapy: Target SpO₂ 88–92%; fan therapy for dyspnea perception
  • NSIP pattern: SSc-ILD is predominantly NSIP histologically — historically more steroid-responsive than UIP/IPF, but at end stage, corticosteroid therapy is not indicated and carries SRC risk
SSc-PAH Management (Card #45 Framework)
Review
  • SSc-PAH responds less well to PAH-targeted therapies than idiopathic PAH — worse outcomes at equivalent hemodynamic severity from combined cardiorespiratory and systemic disease burden[12]
  • Endothelin receptor antagonists (bosentan, macitentan): Continue if tolerated and providing functional benefit. Monitor LFTs with bosentan[13]
  • PDE5 inhibitors (sildenafil, tadalafil): Dual benefit — PAH and Raynaud's/digital ulcers. High comfort value
  • Prostacyclin analogs (epoprostenol, treprostinil): If patient is on IV prostacyclin pump — NEVER interrupt; short half-life means abrupt cessation causes rapid hemodynamic collapse. The pump must be maintained as a clinical emergency protocol at enrollment[14]
  • CCB for PAH: NOT appropriate in SSc-PAH unless vasoreactivity specifically documented at RHC — almost never vasoreactive in SSc
Scleroderma Renal Crisis (SRC)
Grade A
  • Captopril is the EMERGENCY medication for SRC — start 12.5–25 mg q8h immediately upon recognition; titrate aggressively to lower blood pressure[25]
  • Before ACE inhibitors: SRC was uniformly fatal within days. With captopril: renal recovery in ~75% (complete in ~25%, partial in ~50%)[26]
  • SRC recognition protocol: At every SSc hospice visit — if SBP >150 OR creatinine rising in dcSSc or anti-RNAP III patient → assume SRC → start captopril immediately → call rheumatologist
  • Corticosteroid caution: Prednisone >15 mg/day is a documented SRC precipitant. Even in the non-SRC patient, high-dose steroids carry SRC risk[27]
  • Do NOT withhold captopril for rising creatinine — the rising creatinine of SRC is the indication for captopril, not a contraindication
GI Management (Card #58 Framework)
  • GERD/esophageal dysmotility: PPI at adequate dose (omeprazole 40 mg BID or pantoprazole 40 mg BID); head-of-bed elevation; small frequent meals[39]
  • Gastroparesis: Metoclopramide 10 mg 30 min before meals (max 12 weeks per FDA; in hospice context, benefit may outweigh tardive dyskinesia risk if prognosis is short)
  • SIBO (small intestinal bacterial overgrowth): Rotating antibiotics — rifaximin 550 mg TID × 14 days alternating with metronidazole or ciprofloxacin; SIBO is nearly universal in SSc GI involvement[40]
  • Fecal incontinence: Affects up to 38% of SSc patients (internal anal sphincter fibrosis); loperamide, biofeedback, and dignity-preserving management[42]
  • Intestinal failure/TPN: When GI absorption fails — TPN provides nutrition but requires central line and monitoring. Comfort-benefit assessment applies
Raynaud's & Digital Ulcer Management
  • Calcium channel blocker: Nifedipine XL 30–60 mg daily or amlodipine 5–10 mg daily — first-line for Raynaud's[31]
  • Sildenafil 20–25 mg TID: PDE5 inhibitor; dual benefit for PAH and Raynaud's/digital ulcers (Pope et al. RCT)[34]
  • Bosentan for digital ulcer prevention: RAPIDS-1 and RAPIDS-2 trials demonstrated reduction in new digital ulcer formation[32]
  • Iloprost IV: For critical digital ischemia; hospital-administered prostacyclin infusion[35]
  • Digital sympathectomy: Surgical option for refractory critical digital ischemia; consult vascular surgery
  • Wound care: EMLA cream + opioid pre-procedure analgesia protocol before any dressing change
Pain & Musculoskeletal Management
  • Multi-mechanism pain: SSc pain requires simultaneous management of musculoskeletal (joint, tendon), neuropathic (digital ischemia), ischemic (Raynaud's), and visceral (GI) pain[44]
  • Scheduled acetaminophen: 650–1000 mg q6h for musculoskeletal baseline pain (Husebo framework)
  • Gabapentin 100–300 mg TID: Neuropathic pain from digital ischemia — same framework as diabetic neuropathy
  • Opioids: For severe digital ulcer pain, pleurisy, visceral pain. Assess renal function for opioid selection — if GFR <30, avoid morphine (6-MAG accumulation); use hydromorphone or fentanyl[46]
  • Paraffin wax therapy: For hand stiffness and joint contractures — warm paraffin dip improves ROM and reduces pain[47]
  • NO NSAIDs: Categorically contraindicated in SSc — SRC risk
S05Clinician

When Therapy Makes Sense

Evidence-based criteria for continuing disease-directed and symptom-modifying therapy in SSc at hospice enrollment. Some SSc medications are comfort medications.

In SSc, the distinction between "disease-modifying therapy" and "comfort therapy" is not binary. Many SSc medications that were started for disease modification provide direct ongoing symptom benefit. The captopril that prevents scleroderma renal crisis is a life-saving intervention even in a hospice patient. The vasodilator that reduces digital ischemia provides daily comfort. The PPI that suppresses GERD prevents the aspiration that worsens the fibrotic lungs. The hospice clinician must evaluate each medication individually rather than applying a blanket discontinuation approach.[7]

  1. 01
    Captopril for SRC recognition and treatment: Start immediately for any suspected scleroderma renal crisis — rising creatinine + hypertension in an anti-RNAP III positive SSc patient is SRC until proven otherwise. SRC is a reversible emergency that kills within days without treatment. This is a non-negotiable standing order at hospice enrollment.[25]
  2. 02
    Pre-procedure analgesia for digital ulcer wound care: EMLA cream under occlusion 45–60 min before wound care; opioid 30–45 min before wound care; PAINAD score before and during. These are standing clinical orders, not suggestions — the fingertip is the most densely innervated surface of the body.[45]
  3. 03
    Vasodilator therapy for Raynaud's: Continue calcium channel blocker (nifedipine or amlodipine) and sildenafil if already in regimen. Vasodilation that reduces digital ischemia provides direct comfort benefit. These are comfort medications in SSc.[31]
  4. 04
    PPI at adequate dose for GERD: Omeprazole 40 mg daily or pantoprazole 40 mg daily minimum. The GERD that is not adequately suppressed causes esophagitis, regurgitation, and aspiration worsening pulmonary fibrosis. If PPI at enrollment is at an inadequate dose, increase it.[39]
  5. 05
    Prokinetic for gastroparesis: Metoclopramide before meals continues at hospice enrollment. The GI dysmotility of SSc produces nausea, vomiting, bloating, and malnutrition that are primary quality-of-life burdens. Same GI management framework as Card #58.[41]
  6. 06
    Neuropathic pain management for digital neuropathy: Gabapentin at established dosing for the neuropathic pain and allodynia from Raynaud's ischemia. Same gabapentin framework as diabetic neuropathy (Card #48).[44]
  7. 07
    Scheduled acetaminophen for musculoskeletal pain: Tendon friction rub pain, arthralgia, and myalgia of SSc respond to the Husebo scheduled acetaminophen 650–1000 mg q6h framework. This is baseline analgesia in SSc.
  8. 08
    Opioids for severe multi-mechanism pain: Digital ulcers, pleurisy from SSc-ILD, abdominal pain from gut dysmotility. Assess renal function for opioid selection — captopril-treated SRC patients may have fluctuating GFR. If GFR <30 mL/min: avoid morphine; use hydromorphone or fentanyl.[46]
  9. 09
    PAH-targeted therapy continuation: If the patient is on sildenafil, bosentan, or prostacyclin analogs — do not discontinue without careful assessment. The hemodynamic consequences of abrupt prostacyclin discontinuation are life-threatening. Sildenafil and bosentan discontinuation should be gradual with monitoring.[14]
  10. 10
    SIBO rotating antibiotic protocol: The bacterial overgrowth that causes bloating, diarrhea, and malabsorption responds to rotating antibiotics. Continuing this at hospice provides direct GI comfort.[40]
S06Clinician

When It Doesn't

Knowing when treatment stops helping is not clinical failure. In SSc, several medications are actively dangerous and must be stopped or avoided.

SSc has specific medication contraindications that are not shared by most other hospice diagnoses and that produce irreversible harm if missed. The three non-negotiable safety acts at hospice enrollment are: no NSAIDs, no high-dose corticosteroids, and SRC monitoring at every visit. Beyond these, the assessment of disease-modifying therapy in end-stage SSc requires recognizing that the fibrosis that these medications were slowing is already established and irreversible — the ongoing burden of immunosuppression may exceed any residual benefit.[27]

  1. 01
    High-dose corticosteroids in any SSc patient: The most dangerous medication in SSc. Prednisone >15 mg/day is a documented precipitant of scleroderma renal crisis. Even if steroids are needed for a complication (ILD exacerbation, myositis, serositis), use the lowest effective dose and monitor blood pressure and creatinine daily. Document the SRC risk of any corticosteroid use in the SSc care plan.[27]
  2. 02
    NSAIDs for any pain indication in SSc: NSAIDs reduce renal prostaglandins that maintain renal arteriolar perfusion — the same mechanism as in ESLD (Card #49) and SCD (Card #57). In SSc where renal crisis is the most feared acute complication, NSAID use adds renal risk that is categorically unacceptable. Acetaminophen and opioids are the appropriate analgesics. Post this contraindication as prominently as the morphine prohibition in ESRD.[25]
  3. 03
    Calcium channel blockers for PAH in SSc (unless vasoreactivity specifically documented at RHC): Unlike idiopathic PAH where CCBs are effective in the 10–15% who are vasoreactive, SSc-PAH is almost never vasoreactive. High-dose nifedipine or diltiazem used for PAH can cause hypotension and clinical deterioration. Note: low-dose nifedipine for Raynaud's IS appropriate — it is the high-dose PAH-indication use that is contraindicated.[12]
  4. 04
    Immunosuppressant escalation at end stage: Mycophenolate, rituximab, and cyclophosphamide that were reducing fibrosis progression in early-to-middle SSc have diminishing benefit as disease reaches end stage. They add immunosuppression-related infection risk (PCP, Aspergillus, opportunistic infections) without proportional benefit in a patient whose fibrosis is established. Reassess comfort-benefit at enrollment.[55]
  5. 05
    Prophylactic ACE inhibitors without SRC: The historical practice of prophylactic ACE inhibitor therapy in SSc to "prevent" SRC has been disproven — ACE inhibitors given prophylactically do NOT prevent SRC and may mask the blood pressure elevation that is the earliest warning sign, delaying recognition. ACE inhibitors should be used only as TREATMENT of active SRC, not prophylaxis.[28]
  6. 06
    Aggressive nutritional supplementation overriding patient preference: The SSc patient with end-stage GI failure who has stopped tolerating oral intake should not be subjected to forced feeding or TPN initiation that conflicts with comfort goals. When GI absorption has failed, the decision about TPN is a goals-of-care conversation, not a nutritional intervention.[43]
  7. 07
    Nintedanib continuation when GI side effects exceed pulmonary benefit: Nintedanib causes diarrhea and nausea in many patients. In end-stage SSc where the GI tract is already dysmotile and the patient is already malnourished, adding a medication that worsens diarrhea requires explicit comfort-benefit assessment. Discontinuation is appropriate when the GI burden exceeds any perceived pulmonary benefit.[7]

📋 Clinician note

SSc patients are among the most under-referred to palliative care and hospice of any rheumatological disease. The chronic, multi-system nature of SSc produces a "boiling frog" pattern where each organ declines gradually and no single specialist feels empowered to initiate the hospice conversation. The rheumatologist is managing the disease, the pulmonologist is managing the lungs, the gastroenterologist is managing the gut — and nobody is managing the whole patient. The hospice clinician may be the first provider who looks at the entire picture and names the reality: this patient has end-stage multi-organ disease from SSc, and the focus should now be comprehensive comfort care.

S07Everyone

Out-of-the-Box Approaches

Evidence-graded integrative, interventional, and complementary approaches for SSc at end of life. Grade A = RCT; B = multi-observational/meta-analysis; C = limited clinical; D = expert opinion.

SRC Recognition & Captopril Protocol
Grade A
Captopril 12.5–25 mg PO q8h → titrate q12h to lower BP by 20 mmHg/24h
The most urgent clinical safety act in SSc hospice. Before ACE inhibitors (Steen & Medsger, late 1980s–1990s), SRC was rapidly fatal. With aggressive captopril therapy, renal recovery is achievable in ~75%. Steen et al. 1990 (Annals of Internal Medicine) demonstrated that delay in initiating captopril directly correlated with worse renal outcomes. At every SSc hospice visit: measure BP; if SBP >150 OR creatinine rising in dcSSc/anti-RNAP III patient → assume SRC → start captopril immediately → call rheumatologist. The hospice clinician who correctly identifies SRC has potentially prevented permanent ESRD in a patient with meaningful quality of life remaining.[25][26]
EMLA + Opioid Pre-Procedure Analgesia for Digital Ulcers
Grade A
EMLA cream 2.5% lidocaine/2.5% prilocaine under occlusion 45–60 min pre-procedure + opioid PO/SQ 30–45 min pre-procedure
Dual-mechanism analgesia for the most densely innervated wound site on the body. EMLA has Grade A evidence from RCTs for topical procedural analgesia. Opioid pre-medication has Grade A evidence for procedural pain management. Combined, they make fingertip wound care tolerable in a body with neuropathic sensitization from years of Raynaud's ischemia. The PAINAD score before and during wound care documents the pain management effectiveness. Without pre-procedure analgesia, SSc digital ulcer wound care causes systematic preventable suffering.[45]
Paraffin Wax Therapy for Hand Function
Grade B
Paraffin wax dip at 52–54°C, 6–10 layers, wrap 15–20 min, followed by gentle ROM exercises 3–5×/week
Evidence from multiple observational studies and small RCTs in SSc demonstrates that paraffin wax therapy improves hand function, reduces stiffness, and decreases pain. The warmth delivers vasodilation to the hands (beneficial in Raynaud's) and the paraffin maintains contact heat that softens the fibrotic skin. Followed by gentle range-of-motion exercises, this is a non-pharmacological comfort intervention that addresses hand disability, a primary SSc quality-of-life concern. Can be done by family caregivers at home after training.[47]
Fan Therapy for SSc-ILD Dyspnea
Grade A
Handheld fan directed at face, T2/T3 trigeminal dermatome, during dyspnea episodes
The trigeminal nerve cold-air reflex reduces the perception of breathlessness. Multiple RCTs (Galbraith et al. 2010, Bausewein et al.) demonstrate fan therapy reduces dyspnea severity in chronic lung disease. In SSc-ILD, this is a non-pharmacological adjunct to opioids and oxygen that families can provide at any hour. The fan does not replace opioids — it supplements them. Position the fan to direct airflow across the nasal/perioral area.[9]
Oral Stretching Exercises for Microstomia
Grade B
Oral augmentation exercises: TheraFlex® Microstomia Prevention System or wooden tongue depressor stacking; 5–10 min 2–3×/day
SSc microstomia progressively restricts oral aperture, affecting nutrition, dental care, speech, and emergency airway access. Multiple observational studies document that structured oral stretching exercises slow the rate of oral aperture reduction and may maintain or slightly improve opening. In the hospice context, this is a dignity and function intervention — maintaining the ability to eat, to speak clearly, and to receive oral medications. Document baseline oral aperture in cm at enrollment and reassess monthly.[50]
Warm Room Protocol for Raynaud's Prevention
Grade B
Ambient room temperature 70–75°F (21–24°C); heated gloves/mittens; chemical hand warmers; insulated socks; avoid cold drafts
Environmental temperature management is the single most impactful non-pharmacological intervention for Raynaud's in SSc. Core body warming prevents digital vasospasm more effectively than local hand warming alone. In the home hospice setting: maintain ambient temperature above 70°F; use heated gloves or chemical hand warmers during cold months; avoid air conditioning draft on extremities; warm IV fluids before infusion. Family education about the temperature-Raynaud's connection prevents avoidable pain episodes.[31]
Dignity Therapy for Identity Grief
Grade B
Structured protocol: 1–2 sessions (30–60 min) with trained facilitator; recorded, transcribed, edited, and returned as "generativity document"
Dignity therapy (Chochinov et al. 2005 JCO) is a structured life narrative intervention that reduces suffering and increases sense of meaning in terminally ill patients. In SSc, where identity grief from progressive physical transformation is a primary psychosocial burden, dignity therapy provides a framework for the patient to record her story — who she was before, who she became through the disease, and what she wants to leave behind. The generativity document becomes a legacy for the family.[48]
Biofeedback for Raynaud's Vasospasm
Grade C
Thermal biofeedback: 8–12 sessions training digital temperature awareness and voluntary peripheral vasodilation
Thermal biofeedback teaches patients to increase digital blood flow through learned vasodilatory responses. Limited clinical evidence in SSc (stronger in primary Raynaud's) suggests modest reduction in attack frequency and severity. In the hospice context, this is most appropriate for patients with early-to-moderate Raynaud's who have the cognitive capacity and time to complete training. Not appropriate in the final weeks — this is an earlier hospice intervention.[31]
Music Therapy for Procedural Pain & Anxiety
Grade B
Patient-selected music during wound care, during Raynaud's attacks, and at bedtime; 30–60 min sessions
Meta-analyses demonstrate that music therapy reduces procedural pain, anxiety, and opioid requirements across multiple clinical settings. In SSc, where wound care is frequent and painful and anxiety about the next Raynaud's attack is constant, music therapy provides an accessible non-pharmacological complement to the EMLA/opioid protocol. Particularly effective when patient selects the music. Can be provided by trained music therapists or by family caregivers with guidance.[48]
Topical Nitroglycerin for Digital Ulcers
Grade C
NTG 2% ointment applied to affected digit proximal to ulcer; 1/4 inch ribbon BID; monitor for headache and hypotension
Topical nitroglycerin is a local vasodilator that increases digital blood flow. Small case series and pilot studies suggest it may promote digital ulcer healing and reduce Raynaud's severity when applied locally. The evidence is limited by small sample sizes and lack of RCTs. In the hospice context, it is a reasonable trial when systemic vasodilators are maximized and digital ischemia persists. Monitor for headache (most common side effect) and systemic hypotension. Do not use with concurrent PDE5 inhibitor due to additive hypotension risk.[35]
S08Everyone

Natural & Herbal Options

Evidence grading, dosing, drug interaction flags, and SSc-specific contraindications. Patients will use supplements — this section helps you have the right conversation.

From the Field
Waldo Rios, NP
Hospice NP · 12+ Years
"SSc patients have been managing a chronic disease for years — many of them have tried every supplement their rheumatologist, their naturopath, and their neighbor have suggested. The conversation is: 'I want to know everything you're taking — not to judge, but because some supplements interact with your sildenafil, your bosentan, and your blood pressure medications in ways that matter.' The SSc supplement landscape has four specific traps: vasoconstrictive supplements that worsen Raynaud's, CYP3A4 interactions with bosentan and sildenafil, fibrosis-modulating supplements with unpredictable effects, and the GI absorption uncertainty from dysmotility."
— Waldo, NP
Herb / Supplement Evidence Grade Typical Dose Potential Benefit ⚠ Interactions / Contraindications
Fish Oil (Omega-3)Grade B1–3 g EPA+DHA dailyAnti-inflammatory; mild vasodilatory effect via PGI3 production; small trials in SSc show modest reduction in Raynaud's attack frequency/severityMild antiplatelet effect — monitor if on anticoagulation; GI upset may worsen SSc dysmotility at high doses; absorption may be erratic in SSc gastroparesis
Vitamin DGrade B1000–4000 IU daily (titrate to serum 25-OH-D 30–50 ng/mL)Vitamin D deficiency is common in SSc (reduced sun exposure, malabsorption); may have immunomodulatory effects; supports bone health (AVN risk in SSc from vasculopathy and steroids)High doses (>10,000 IU/day) may promote calcinosis in SSc through hypercalcemia — a specific SSc concern; check serum calcium; supplement to sufficiency, not excess
ProbioticsGrade BMulti-strain probiotic; Lactobacillus/Bifidobacterium 10–50 billion CFU dailySSc GI dysbiosis and SIBO contribute to bloating, diarrhea, malabsorption; probiotics may improve GI symptoms as adjunct to rotating antibioticsIn immunosuppressed SSc patients (on mycophenolate, rituximab), rare risk of probiotic bacteremia/fungemia; generally safe in immunocompetent SSc patients
Ginkgo bilobaGrade C120–240 mg standardized extract dailyPeripheral vasodilatory properties; small pilot studies in Raynaud's suggest modest reduction in attack severity; PAF antagonist may reduce platelet aggregationAntiplatelet effect — bleeding risk with anticoagulants; CYP3A4 inducer — may reduce sildenafil and bosentan levels; absorption uncertain in SSc
Coenzyme Q10Grade C100–300 mg dailyAntioxidant; may support cardiac function in SSc cardiomyopathy; mitochondrial support in the setting of myocardial fibrosisMild blood pressure reduction may be additive with vasodilators; absorption may be reduced in SSc GI dysmotility; generally well-tolerated
Turmeric/CurcuminGrade C500–1500 mg curcumin extract daily with piperine for absorptionAnti-inflammatory; preclinical data shows inhibition of TGF-β signaling (the master fibrosis pathway in SSc); may have anti-fibrotic properties in vitroCYP3A4 and CYP2C9 inhibitor — may increase sildenafil and bosentan levels; the anti-fibrotic signal is preclinical only — no SSc clinical trials; GI upset may worsen dysmotility; absorption is poor and further reduced in SSc
Evening Primrose Oil (GLA)Grade C3–6 g daily (providing ~270–540 mg GLA)Gamma-linolenic acid (GLA) is an omega-6 with anti-inflammatory prostaglandin production; small studies in Raynaud's/SSc suggest modest vascular benefitAntiplatelet effect; seizure threshold reduction (theoretical — relevant if concurrent brain metastases or CNS involvement); absorption erratic in SSc
N-Acetylcysteine (NAC)Grade C600–1200 mg dailyAntioxidant; mucolytic (may help with SSc-ILD secretions); small studies show improvement in digital ulcer healing and Raynaud's outcomesMay cause GI upset (nausea, diarrhea) worsening SSc dysmotility; generally well-tolerated; no significant drug interactions with SSc medications
🚫 Avoid in Systemic Sclerosis
  • Ephedra/Ma Huang: Potent sympathomimetic vasoconstrictor — will worsen Raynaud's phenomenon and digital ischemia; may precipitate hypertensive crisis increasing SRC risk; categorically contraindicated in SSc
  • Licorice root (glycyrrhizin): Produces pseudoaldosteronism — sodium retention, potassium wasting, hypertension; may contribute to blood pressure elevation that mimics or triggers SRC; contraindicated in any patient with SRC risk
  • St. John's Wort: Potent CYP3A4 inducer — will dramatically reduce levels of sildenafil, bosentan, and calcium channel blockers (all SSc-essential medications); also reduces immunosuppressant levels if patient is on mycophenolate
  • High-dose Vitamin D (>10,000 IU/day): Risk of hypercalcemia that may worsen calcinosis — a disease-specific SSc concern where calcium deposits already form in soft tissues
  • Decongestant-containing supplements: Pseudoephedrine, phenylephrine, and any sympathomimetic in cold/flu supplements — vasoconstriction worsens Raynaud's and digital ischemia
  • Echinacea: Immune-stimulating properties may theoretically worsen SSc autoimmune activation; contraindicated in patients on immunosuppressants (mycophenolate, rituximab) from immunostimulatory-immunosuppressant conflict
S09Everyone

Timeline Guide

A guide, not a prediction. SSc has the longest chronic disease course of any hospice diagnosis in this series — many patients have had the disease for 15–25 years before reaching hospice.

The SSc timeline is defined by the longest chronic disease course of any hospice diagnosis — many patients have had SSc for 15–25 years before reaching hospice-eligible disease severity. The trajectory involves an early inflammatory phase (rapid skin progression in dcSSc over first 3–5 years, organ involvement begins), a stabilization phase (skin may plateau but internal organs continue accumulating damage), and a late progressive phase (organ failure from accumulated fibrosis — the ILD that has reached the hospice threshold, the PAH that has developed, the gut that can no longer absorb). Subtype, antibody profile, and specific organ involvement dramatically alter the trajectory. Anti-RNAP III dcSSc with SRC has the most acute trajectory; anti-centromere lcSSc with late PAH has the most gradual.[4]

YRS
Years Before Hospice — The Long Disease Course
  • Initial symptom: Raynaud's phenomenon starting in the early 40s; skin tightening of fingers prompting rheumatology referral; months of diagnostic workup (ANA positive, SSc-specific antibodies identified, PFTs ordered, echocardiogram normal at that time)
  • Years of rheumatology management: Multiple medications tried; ILD appearing on CT chest and progressing; PAH screening echos normal then showing rising RVSP; GI symptoms managed with PPI then requiring more intervention
  • Functional losses accumulating: Career requiring fine motor skills lost to hand changes; career requiring physical stamina lost to lung involvement; relationships altered by facial changes and reduced functional capacity
  • Surgeries: Digital amputations for gangrene; joint replacements for AVN from SSc vasculopathy; colostomy for anorectal dysfunction; central line placement for TPN or prostacyclin
  • The patient has been fighting this disease for a decade or more — the hospice clinician enters a long, complex story
MOS
Months — Hospice Enrollment & Stabilization
  • Hospice enrollment triggers: NYHA Class III–IV from SSc-ILD or SSc-PAH; end-stage GI failure requiring TPN; severe malnutrition (albumin <2.5 g/dL); functional decline below PPS 40%; refractory scleroderma renal crisis
  • Comprehensive medication review: Inventory all SSc medications (often 10–15); assess comfort-benefit for each; establish the three non-negotiable safety acts (no NSAIDs, no high-dose steroids, SRC monitoring protocol)
  • Establish the pre-procedure analgesia protocol for digital ulcer wound care as a standing order
  • Baseline assessments: Oral aperture measurement; hand function documentation; digital ulcer inventory; nutritional status; functional status trending
  • Goals of care clarified: The SSc patient has been managing complexity for years — she knows her disease, her body, and her priorities. Listen to what she has learned.
WKS
Weeks — Progressive Decline
  • Increasing dyspnea: SSc-ILD progression reduces reserve further; opioid titration for dyspnea becomes primary management focus; oxygen requirements increase; activity tolerance narrows to bed-to-chair
  • GI failure advancing: Oral intake diminishing; bloating and nausea increasing; weight loss accelerating; oral medication absorption becoming unreliable — transition to SQ or rectal routes
  • Digital ischemia worsening: Raynaud's attacks more frequent despite vasodilators; digital ulcers may worsen; wound care frequency increasing
  • Pain intensifying: Multi-mechanism pain requires opioid escalation; neuropathic component may require gabapentin increase; musculoskeletal pain from immobility
  • Focus: Symptom management intensification; comfort kit in place; family teaching about what to expect; route of medication administration transition (PO → SQ/SL)
DAYS
Days — Active Dying / Pre-Active Phase
  • Bed-bound; minimal oral intake; sleeping most of the day; progressive withdrawal from engagement
  • SSc-specific signs: Raynaud's becoming more persistent (peripheral vasoconstriction from dying process compounds SSc vasculopathy); digital cyanosis deepening; skin mottling may begin at knees/feet but can appear earlier in SSc due to baseline vasculopathy
  • Dyspnea management: Continuous low-dose morphine infusion (SQ or IV) for comfort; oxygen continued for comfort; fan continued; positioning for respiratory ease
  • Discontinue medications that cannot be given SQ: Oral medications are no longer reliable; continue captopril SQ or rectal if SRC risk is active; stop oral SSc medications that have no SQ equivalent (nintedanib, bosentan) — document clinical rationale
  • Family teaching: What the final days look like in SSc — the tight skin and contracted hands are the disease, not pain; the facial rigidity is the disease, not emotional withdrawal; she can hear you even when she cannot respond
HRS
Final Hours
  • Breathing pattern changes: Cheyne-Stokes or agonal breathing; mandibular breathing; terminal secretions — glycopyrrolate 0.2 mg SQ q4h for secretions (preferred over hyoscine in SSc because it does not cross BBB)
  • Peripheral changes: Mottling progresses centrally; digital cyanosis becomes fixed (already present from SSc vasculopathy — explain to family this is worsening of what has been present, not a new sign); skin may feel cool even in the warm room maintained for Raynaud's management
  • Unresponsive or minimally responsive; auditory awareness may persist — family should continue to speak to her, touch her hands gently (avoiding pressure on digital ulcers and calcinosis sites), and be present
  • Emergency medications drawn and labeled at bedside: Morphine for dyspnea crisis; midazolam 2.5–5 mg SQ for terminal agitation; glycopyrrolate for secretions. The SSc patient is unlikely to have hemorrhagic crisis but may have sudden respiratory decompensation from PAH-related right heart failure
  • After death: The skin tightness and hand contractures remain — prepare the family that the body will look as it has looked in life; the rigidity they see is the disease, not rigor mortis (which develops later)
S10Clinician

Medications to Anticipate

SSc-specific pharmacology for comfort. Three non-negotiable safety priorities, multi-system symptom management, and the SSc comfort kit.

🚨 Three Non-Negotiable Safety Priorities at First Visit

  • (1) NO NSAIDs in any SSc patient — NSAIDs reduce renal prostaglandins that maintain renal arteriolar perfusion and add SRC risk. Post this contraindication as prominently as the morphine prohibition in ESRD. Use acetaminophen and opioids for all analgesia.
  • (2) NO HIGH-DOSE CORTICOSTEROIDS — Prednisone >15 mg/day is a documented SRC precipitant. If steroids are required for any indication, use the lowest effective dose and monitor BP and creatinine daily.
  • (3) SRC MONITORING PROTOCOL — Measure blood pressure and note creatinine trend at every visit in any patient with dcSSc or anti-RNAP III antibodies. Rising BP + rising creatinine = start captopril immediately and contact rheumatologist.
DrugClass / Target SymptomStarting DoseNotes / Cautions
CaptoprilACE Inhibitor / Scleroderma Renal Crisis12.5–25 mg PO q8h → titrate aggressivelyTHE emergency medication in SSc. Start immediately for any suspected SRC (rising BP + rising creatinine in dcSSc/anti-RNAP III patient). Do NOT withhold for rising creatinine — that IS the indication. Continue for minimum 12–18 months even if renal recovery appears complete. Monitor BP twice daily during acute SRC.[25]
OmeprazolePPI / GERD & Esophagitis40 mg PO BIDSSc GERD requires high-dose PPI — standard 20 mg daily is inadequate. Esophageal dysmotility + lower esophageal sphincter incompetence produces severe reflux. Inadequately suppressed GERD → esophagitis → aspiration → worsening ILD. Alternative: pantoprazole 40 mg BID.[39]
Nifedipine XLCCB / Raynaud's Phenomenon30–60 mg PO dailyFirst-line vasodilator for Raynaud's. Low-dose for Raynaud's is appropriate (30–60 mg). High-dose for PAH is NOT appropriate in SSc-PAH unless vasoreactivity documented. Monitor for hypotension. Alternative: amlodipine 5–10 mg daily.[31]
SildenafilPDE5 Inhibitor / PAH + Raynaud's + Digital Ulcers20–25 mg PO TIDTriple benefit in SSc: PAH vasodilation, Raynaud's improvement, digital ulcer healing. High comfort value. CYP3A4 substrate — watch for interactions with bosentan (CYP inducer may reduce sildenafil levels). Do not use with topical nitroglycerin (additive hypotension).[34]
MorphineOpioid / Dyspnea + Pain2.5–5 mg PO/SQ q4h PRN; titrate to effectFirst-line for SSc-ILD dyspnea and severe pain. ⚠ If GFR <30 mL/min (SRC, CKD): avoid morphine — 6-MAG accumulation risk. Use hydromorphone or fentanyl instead. Systemic route for dyspnea. Pre-procedure dosing 30–45 min before wound care.[46]
HydromorphoneOpioid / Pain + Dyspnea (renal-safe alternative)0.5–1 mg PO q4h or 0.2–0.4 mg SQ q4hPreferred opioid when renal function is impaired (SRC, CKD with GFR <30). No toxic metabolite accumulation. Titrate to effect. Use for pre-procedure analgesia if morphine is contraindicated.[46]
GabapentinNeuropathic / Digital Neuropathy + Allodynia100–300 mg PO TID; titrate to 900–1800 mg/dayNeuropathic pain from SSc digital ischemia and Raynaud's-related nerve damage. Same framework as diabetic neuropathy. Start low, titrate slowly. Adjust for renal function. Sedation, dizziness, ataxia at higher doses.[44]
AcetaminophenAnalgesic / Musculoskeletal Pain650–1000 mg PO q6h scheduledBaseline analgesia for tendon friction rub pain, arthralgia, myalgia. Husebo scheduled acetaminophen framework. Maximum 3 g/day (2 g/day if hepatic impairment). NO NSAIDs in SSc — acetaminophen is the sole non-opioid systemic analgesic.
MetoclopramideProkinetic / Gastroparesis10 mg PO 30 min before meals + at bedtimeSSc gastroparesis from GI smooth muscle fibrosis. FDA black box: tardive dyskinesia risk with >12 weeks use — in hospice context with limited prognosis, benefit may outweigh this risk. Alternative: domperidone (not FDA-approved in US; available in some settings).[41]
RifaximinAntibiotic / SIBO550 mg PO TID × 14 days; rotate q4–6 wksSSc small intestinal bacterial overgrowth from dysmotility. Rotating antibiotic protocol with alternating cycles of rifaximin, metronidazole, or ciprofloxacin. Non-absorbed — minimal systemic side effects. Reduces bloating, diarrhea, malabsorption.[40]
EMLA CreamTopical Anesthetic / Digital Ulcer Pre-Procedure2.5% lidocaine/2.5% prilocaine; apply under occlusion 45–60 min before wound careStanding order for ALL SSc digital ulcer wound care. Apply to wound margins (not into open wound bed). Cover with occlusive dressing. Combined with systemic opioid pre-medication for dual-mechanism analgesia at the most densely innervated wound site.[45]
BosentanERA / PAH + Digital Ulcer Prevention62.5 mg PO BID × 4 wks → 125 mg PO BIDDual indication in SSc: PAH management and prevention of new digital ulcers (RAPIDS trials). Continue if tolerated and providing benefit. Monitor LFTs monthly (hepatotoxicity risk). CYP3A4/2C9 inducer — reduces sildenafil levels (may need dose adjustment).[32]
LoperamideAntidiarrheal / Fecal Incontinence2–4 mg PO PRN; max 16 mg/daySSc fecal incontinence from internal anal sphincter fibrosis affects up to 38%. Loperamide improves sphincter tone and reduces diarrheal episodes. Use cautiously if significant constipation alternating with diarrhea.[42]
LorazepamBenzodiazepine / Anxiety + Body Image Distress0.5–1 mg PO/SL q4–6h PRNAcute anxiety, Raynaud's-triggered distress, procedural anxiety before wound care. Avoid scheduled use unless breakthrough is frequent. Sublingual route available when oral intake is unreliable.
MidazolamBenzodiazepine / Terminal Agitation2.5–5 mg SQ PRNTerminal agitation and catastrophic symptom management. Have in comfort kit drawn and labeled. SSc-specific: sudden respiratory decompensation from PAH-related right heart failure may require rapid sedation for comfort.
GlycopyrrolateAnticholinergic / Terminal Secretions0.2 mg SQ q4h PRNReduces terminal secretions without CNS effects. Preferred over hyoscine in SSc because it does not cross the blood-brain barrier — important in a patient population where cognitive preservation for family interaction is valued. Anticholinergic effects may worsen GI dysmotility — acceptable trade-off in the final hours.[9]
MirtazapineAntidepressant / Depression + Insomnia + Anorexia7.5–15 mg PO QHSFirst-line antidepressant in SSc hospice — addresses depression, insomnia, and appetite simultaneously. The appetite-stimulant and antiemetic properties are particularly valuable in SSc where malnutrition is a primary concern. Faster onset than SSRIs.

🌿 SSc Symptom Management Decision Tree

Evidence-based · SSc-specific · Hospice-adapted
Select a symptom below to begin
What is the primary symptom to address?

🚨 SSc Comfort Kit Must-Haves

  • Captopril 25 mg tablets: At bedside with written instructions — for SRC emergency. Family and patient must know where it is and when to take it.
  • Morphine or hydromorphone: Concentrated oral solution + SQ syringes drawn and labeled for dyspnea crisis and breakthrough pain
  • EMLA cream + occlusive dressings: Pre-positioned for digital ulcer wound care; application instructions written for family
  • Midazolam 2.5–5 mg SQ: Drawn and labeled for terminal agitation or catastrophic respiratory event from PAH decompensation
  • Glycopyrrolate 0.2 mg SQ: For terminal secretions
  • Lorazepam 1 mg SL: For acute anxiety, Raynaud's-associated distress
  • Blood pressure cuff: Essential equipment at bedside — SRC monitoring is a daily clinical act
  • Chemical hand warmers: For Raynaud's attack management — non-pharmacological first response
S11Clinician

Clinician Pointers

High-yield clinical pearls for the SSc hospice team. The things not in the textbook — learned at the bedside over years of caring for a disease that turns the body to stone.

1
Measure blood pressure and check creatinine trend at every visit before any other assessment
The anti-RNAP III positive dcSSc patient who presents with rising blood pressure and rising creatinine has scleroderma renal crisis until proven otherwise. The clinical instinct that says "they're hospice, this is just disease progression" will result in avoidable ESRD in a patient who otherwise has meaningful quality of life remaining. Start captopril, call the rheumatologist, document the SRC recognition as a clinical emergency. The blood pressure check in SSc is not a vital sign — it is a screening act for a reversible emergency.[25]
2
Post the NSAID contraindication at the same visibility as morphine in ESRD
Write it at the top of the medication list, in the supply notes, on the care plan cover page: "NSAIDs ARE CONTRAINDICATED IN SSc — NEVER USE IBUPROFEN, NAPROXEN, KETOROLAC, OR DICLOFENAC — RISK OF SCLERODERMA RENAL CRISIS." The nurse practitioner covering at 2 AM who sees an SSc patient in pain and reaches for ketorolac has made a potentially catastrophic prescribing error. The documentation that prevents this is the care plan that makes the prohibition unavoidable to read.[25]
3
Establish the digital ulcer pre-procedure analgesia as a standing order before you leave the first visit
EMLA cream under occlusion 45–60 minutes before wound care. Opioid 30–45 minutes before wound care. PAINAD score before and during wound care. These are clinical orders, not suggestions. The hospice nurse who changes an SSc digital ulcer dressing without pre-procedure analgesia has caused systematic preventable pain at one of the most densely innervated wound locations in the human body. Write the order. Post it. Make it unavoidable.[45]
4
Assess the oral aperture at enrollment and document the measurement in centimeters
The microstomia that has progressed to 20–25 mm has implications for: dental care (document for the dentist), nutrition (small bolus requirements), emergency airway management (document for any emergency responder), oral medication administration, and oral stretching exercises that slow further progression. The hospice clinician who does not assess and document the oral aperture has missed a functional and dignity assessment that is specific to SSc and that no other clinical setting typically performs.[50]
5
Inventory every digital ulcer at enrollment with location, size, depth, and infection status
The digital ulcer on the right index fingertip that has been present for 4 months and has exposed bone is a different wound from the new ulcer on the left ring finger that started 2 weeks ago. Each requires its own wound care plan. The inventory creates the map for the wound care protocol and the pre-procedure analgesia schedule. Photograph each ulcer at enrollment with consent for wound tracking.
6
If the patient is on IV prostacyclin (epoprostenol/treprostinil) — establish the pump emergency protocol at enrollment
The prostacyclin pump in SSc-PAH must NEVER be interrupted. The short half-life of epoprostenol means abrupt cessation produces rapid pulmonary hypertensive crisis and hemodynamic collapse within minutes. At enrollment: identify the pump, the infusion site, the backup cassettes, the emergency contact for pump malfunction, and the covering clinician authorized to troubleshoot. Document this as a clinical emergency protocol, not a routine medication note.[14]
7
Assess the SSc patient's relationship with her own changed body at the first visit
SSc produces the most visible physical transformation of any disease in this series. The face that has changed, the hands that have contracted, the skin that has tightened — the patient has been watching her body become progressively less recognizable as her own. This is an identity grief that is specific to SSc and that no other clinical framework typically addresses. The hospice social worker or chaplain who asks "what has it been like to watch your body change over these years?" opens a therapeutic conversation that is both assessment and intervention.[48]
8
The SSc patient knows her disease — listen before you teach
This patient has been managing SSc for a decade or more. She has seen more rheumatologists than you have. She knows which vasodilator works for her Raynaud's, which PPI dose controls her reflux, and which position helps her breathe. The hospice clinician who arrives and starts teaching a disease that the patient has lived with for 15 years will lose credibility immediately. Start by listening: "Tell me what you've learned about managing your scleroderma." Then build the comfort plan on her expertise.
9
Recognize that Black women with SSc may have had less access to specialty care
Black women develop SSc at younger ages with more severe organ involvement and shorter survival — a disparity from both biological factors and healthcare access. The Black woman with SSc in your care may have had fewer rheumatology visits, fewer subspecialty referrals, and later diagnosis than a white counterpart. The hospice clinician who recognizes this disparity and provides the comprehensive, coordinated, person-centered care that may not have been consistently available before hospice enrollment is practicing equity at the bedside.[56]
10
The warm room is a medication in SSc
Ambient temperature management is as important as any vasodilator in Raynaud's management. At enrollment, assess the home heating situation: can the room be maintained at 70–75°F? Are there drafts from windows or air conditioning? Does the patient have heated gloves and chemical hand warmers? Is the oxygen humidified (dry O₂ can contribute to facial skin tightness)? The clinical team that ensures the room is warm and the hands are protected has reduced Raynaud's attack frequency more effectively than adding another medication.
From the Field
Waldo Rios, NP
Hospice NP · 12+ Years
"The first time I cared for an SSc patient at hospice, I treated her like a COPD patient with skin problems. I was wrong. This is a disease where the blood pressure at every visit is a screening test for a kidney emergency, where the fingertip wound care requires analgesia 45 minutes before you touch the dressing, where the medication list has interactions that will surprise you, and where the patient sitting in front of you has been fighting a disease that most people have never heard of for longer than some of your newer nurses have been alive. Listen first. Then act."
— Waldo, NP · Terminal2
S12EveryoneClinician

Psychosocial & Spiritual Care

Identity grief, body image transformation, the invisible disease burden, and the specific psychosocial dimensions of SSc at end of life.

Systemic sclerosis produces the most visible physical transformation of any disease in this series — and some of the deepest identity grief. The patient has watched her face change over years — the skin tightening, the oral aperture narrowing, the lips thinning, the facial expression reducing. Her hands have contracted, developed calcinosis nodules, had ulcers on the fingertips. The body that carried her through the first half of life has become progressively different from the body she knew. This is not vanity. This is the fundamental human experience of watching your own physical identity transform against your will — and it produces a grief that is distinct from the grief of dying.[48]

The invisible burden compounds the visible one. The community around the SSc patient may see the skin changes and the hand changes but cannot see the lungs that are fibrosed, the gut that cannot move properly, the blood vessels that are obliterated. The visible changes produce social responses (stares, questions, the awkwardness of others) while the invisible changes produce the functional limitation, the fatigue, the dyspnea, and the malnutrition that are the primary quality-of-life burdens. The SSc patient has been navigating both dimensions simultaneously for years.[51]

The Body That Has Become a Stranger — Identity Grief
Physical Transformation & Identity
  • Facial changes: The face that has changed from the face she knew — tight skin, reduced expression, thinned lips, microstomia. She has watched this transformation happen year by year
  • Hand changes: Contractures, calcinosis nodules, sclerodactyly, digital ulcers, amputations. Hands are identity — they are how we touch, work, create, express
  • Opening the conversation: "What has it been like to watch your body change over these years?" creates space for a relationship between the patient and her changed body that is both assessment and intervention
  • The mirror: Many SSc patients have a complicated relationship with mirrors. Some avoid them. Some study them. Ask. This is a therapeutic entry point.
The Predominantly Female Experience
  • SSc is 4–9:1 female — the physical transformation occurs in a cultural context where women's appearance is socially weighted and scrutinized
  • Intimate relationships: Skin tightening, hand contractures, microstomia, GI symptoms, and fatigue all affect physical intimacy. Assess sexual health at enrollment — most clinicians never ask
  • Motherhood and caregiving: SSc often begins during child-raising years — the mother whose hands can no longer braid her daughter's hair, who cannot open jars, who cannot play the way she used to
  • Social isolation: The visible changes produce avoidance of social situations; the fatigue produces withdrawal; the complex medical schedule produces logistical isolation
Psychological Distress Screening
Depression in SSc — Screen Every Patient
Grade B

Depression prevalence in SSc is 36–65% — significantly higher than most chronic diseases, driven by the combination of chronic pain, disability, body image distress, and the progressive nature of the disease.[51]

  • Single-question screen: "Are you depressed?" — 100% sensitivity in terminally ill populations
  • PHQ-2: "Little interest/pleasure" + "Feeling down/hopeless" — score ≥3 warrants full PHQ-9
  • Mirtazapine 7.5 mg QHS: First-line in SSc hospice — addresses depression, insomnia, and anorexia simultaneously. The appetite-stimulant effect is particularly valuable in the malnourished SSc patient
  • Distinguish depression from appropriate sadness about a progressive disfiguring disease — both deserve attention; only one warrants pharmacotherapy
Anxiety & Body Image Distress
Grade B
  • Anticipatory anxiety: The next Raynaud's attack, the next wound care, the next blood pressure check that might reveal SRC — SSc patients live with multiple anticipatory anxieties simultaneously
  • Body image distress: Distinct from depression — the distress of physical transformation. Assess with: "How do you feel about the changes in your body?" and "What bothers you most about how you look now?"
  • Lorazepam 0.5 mg PRN for acute anxiety; dignity therapy for identity distress; cognitive-behavioral approaches for anticipatory anxiety if cognitive capacity allows
  • Refer to social work and chaplain at enrollment — the psychosocial burden of SSc is too complex for a single clinician
Spiritual Assessment

The spiritual needs of the SSc patient may center on meaning-making from a disease that has progressively taken function and appearance — "Why me?" and "What was the purpose of all this suffering?" are spiritual questions that deserve spiritual engagement. Use the FICA framework: Faith/beliefs, Importance, Community, Address. Ask: "What gives you strength during this time?" In SSc, also ask: "What has this disease taught you?" — many SSc patients have developed profound resilience and wisdom from managing a chronic disease for decades, and that wisdom deserves to be named and honored.

Clinical Pearl

"The SSc patient who has been managing this disease for 20 years has a spiritual life shaped by chronic illness in ways that a patient with a 6-month cancer diagnosis does not. She has had decades to wrestle with 'why me,' to develop coping strategies, to find meaning in suffering. The chaplain who recognizes this depth of experience and asks 'what has this journey taught you about yourself?' honors both the suffering and the wisdom that emerged from it."

  1. 01
    Ask about faith community explicitly: "Is there a faith community or spiritual leader who should know you're ill?" SSc patients may have been too physically limited to attend in-person worship for years — the faith community may not know the severity of the illness.
  2. 02
    Legacy work is particularly meaningful in SSc: The patient whose hands can no longer write may want to dictate letters. The patient whose face has changed may want photographs from before the disease to be displayed alongside current photos. The dignity therapy generativity document captures the whole person — not just the disease.
  3. 03
    Address body donation and autopsy: SSc patients may wish to donate their body to SSc research — the Scleroderma Foundation can facilitate this. This gives meaning to the disease and contributes to understanding for future patients. Discuss before the final days.
  4. 04
    Unfinished business: Relationship ruptures caused or worsened by the disease — the partner who left when the disease changed her appearance, the job that was lost, the activities abandoned. These losses may surface as the patient approaches the end and deserve acknowledgment.
Goals-of-Care Communication in SSc
Opening the Conversation
  • "What is your understanding of where things stand with your scleroderma?" — assesses illness understanding; the SSc patient often has sophisticated disease knowledge
  • "What are you hoping for at this point?" — surfaces values; SSc patients may hope for pain control, hand function preservation, or simply the ability to eat without pain
  • "What are you most afraid of?" — in SSc, common fears include: suffocation (from ILD/PAH), loss of remaining hand function, becoming a burden, the disease taking her face further
  • "What has been the hardest part of living with scleroderma?" — opens the 20-year narrative that frames all goals
SSc-Specific Communication Pearls
  • Do not flinch from the physical changes: The SSc patient is acutely aware of how people react to her appearance. The clinician who maintains normal eye contact and touches her hands without hesitation communicates acceptance
  • Name the disease accurately: "Scleroderma" or "systemic sclerosis" — not "that skin thing." The patient's disease has a name and she has lived with it for decades
  • Acknowledge the marathon: "You've been managing this disease for a long time" validates the years of effort and resilience that preceded hospice enrollment
  • Don't conflate hospice with giving up the fight: The SSc patient has been fighting for years. Frame hospice as: "We're adding a whole team focused entirely on your comfort"
Suicidal Ideation & Hastened Death

Passive wish for death ("I'm ready to go") is common in end-stage SSc and often contextually appropriate in a patient who has lived with progressive physical transformation and multi-organ failure for decades. Assessment requires careful distinction: passive wish for death (common, often appropriate), active suicidal ideation with plan (requires immediate psychiatric engagement — screen specifically for access to means, including hoarded medications from the complex SSc regimen), and medical aid in dying requests (legal in some jurisdictions). The SSc-specific risk factor for suicidal ideation is body image distress — the progressive physical transformation combined with pain and disability creates a specific risk profile that should be assessed directly.

From the Field
Waldo Rios, NP
Hospice NP · 12+ Years
"I had an SSc patient who hadn't looked in a mirror in three years. She told me this on the third visit — not the first. She said, 'The last time I looked, I couldn't find myself in there.' That's not a dermatological complaint. That's an existential crisis wearing a skin disease as a costume. The hospice chaplain who opened that door — who asked 'what has it been like to watch your body change?' — unlocked a grief that she had been carrying alone for years because nobody in a clinic had ever asked."
— Waldo, NP · Terminal2
S13FamilyEveryone

Family Guide

Plain language for families. Share, print, or read aloud at the bedside.

Your loved one has systemic sclerosis (scleroderma) — a disease that affects the skin, lungs, heart, digestive system, kidneys, and blood vessels simultaneously. What you see on the outside — the changed skin, the changed hands, the changed face — is the most visible part of a disease that has been working inside the body for years. Each system affected has its own symptoms and its own management, which is why the care plan for scleroderma is complex. You are an essential part of this care team, and this guide will help you understand what to watch for, what to do, and when to call us.

Próximamente en español. — Coming soon in Spanish.

🚨 SCLERODERMA RENAL CRISIS — A MEDICAL EMERGENCY YOU MUST KNOW

This is the most important section of this guide. Certain patients with scleroderma can develop a serious kidney emergency called scleroderma renal crisis (SRC). It happens quickly and can be reversed if treated immediately — but it can cause permanent kidney failure if missed.

  • Check daily: Blood pressure above 150/100 mmHg; headache combined with rising blood pressure; confusion or increased confusion; creatinine rising on latest labs
  • If you see any of these — do these three things in order:
    1. Give captopril [dose as prescribed] immediately — the tablet is at [location determined at enrollment]
    2. Check blood pressure again in 1 hour
    3. Call the hospice nurse immediately after giving the captopril
  • Do not wait to call the doctor before giving the captopril — giving it immediately is what saves the kidneys.
What You May See
  • Raynaud's attacks: Fingers turning white, then blue, then red, with pain. This happens when exposed to cold or stress. It can look alarming — the fingers may look waxy white then deep blue — but this is a known part of scleroderma, not a new emergency. Follow the Raynaud's protocol below.
  • Digital ulcers: Open sores on the fingertips that look raw and painful. They heal very slowly. Do not touch or change dressings without following the pain medication protocol first — the fingertips are extremely sensitive.
  • Shortness of breath: This may come and go or be constant. It is caused by scarring in the lungs and/or high pressure in the blood vessels of the lungs. Positioning upright, using the oxygen, and the fan directed at the face all help.
  • Difficulty eating: Heartburn, bloating, nausea, feeling full after a few bites, and sometimes vomiting. The digestive system moves slowly in scleroderma. Small frequent meals, keeping the head elevated, and the medications prescribed for digestion all help.
  • Skin tightness and hand stiffness: The tight skin and stiff joints are the disease — they are not caused by pain or cold alone. Gentle range-of-motion exercises and warm paraffin dips can help maintain some flexibility.
  • Facial changes: The tight skin around the mouth and face may make your person look different from how you remember them. The reduced facial expression is the disease affecting the skin — it does not mean they are not feeling emotions. They feel everything; the face just cannot express it as fully.
  • Calcinosis: Hard calcium lumps under the skin, often at the fingertips, elbows, or knees. These can sometimes break through the skin and drain a white chalky material. This is not an infection — it is calcium. Keep the area clean and covered.
How You Can Help
  • During a Raynaud's attack: Move your person to a warm room. Place chemical hand warmers (never directly on skin — wrap in a cloth) near the affected hands. Offer warm (not hot) beverages. Do NOT run the hands under hot water — the SSc skin cannot sense temperature accurately and burns can occur. Do NOT rub or massage the affected fingers — the tissue is ischemic and pressure can cause injury.
  • Keep the room warm: 70–75°F (21–24°C). Avoid cold drafts from windows and air conditioning. The warm room is as important as any medication for preventing Raynaud's attacks.
  • Before wound care: Apply EMLA cream to the fingertip as instructed, cover with the dressing, wait 45–60 minutes. Give the pain medication 30–45 minutes before the nurse arrives for wound care. This preparation makes the wound care tolerable.
  • Small frequent meals: Offer small amounts every 2–3 hours rather than three large meals. Keep the head of the bed elevated 30–45 degrees after eating. Avoid foods that worsen reflux (spicy, acidic, fatty). Do not force food — if your person says they're full after a few bites, that is the disease affecting the stomach, not lack of effort.
  • For shortness of breath: Position upright (head of bed elevated); direct a fan at the face from about 12 inches away; ensure oxygen is flowing at the prescribed rate; give the prescribed morphine or hydromorphone if breathing distress is severe — the nurse has taught you when and how.
  • Touch and presence: Touch the areas that are not affected by ulcers or calcinosis — the forearms, the shoulders, the hair. Be present without needing to fix things. Silence and gentle touch are profoundly therapeutic.
  • The NSAID rule — please memorize this: NEVER give your person ibuprofen (Advil, Motrin), naproxen (Aleve), or any similar pain reliever. These medications can trigger a dangerous kidney crisis in scleroderma. If there is pain, use acetaminophen (Tylenol) or the prescribed opioid. If in doubt, call the nurse first.
📞 Call the hospice nurse immediately if you see:

Blood pressure above 150/100 mmHg (give captopril first, then call) · Sudden worsening of shortness of breath that does not improve with positioning and prescribed medications · New confusion or change in alertness combined with high blood pressure · A finger that turns completely black or blue and does not recover color after warming (may indicate critical ischemia or gangrene requiring urgent assessment) · Fever above 101°F combined with redness or pus around a digital ulcer (possible osteomyelitis or cellulitis) · Vomiting blood or dark coffee-ground material (possible esophageal or GI bleeding from severe esophagitis) · Sudden severe abdominal pain with distension (possible bowel pseudo-obstruction) · Any fall or injury — SSc skin is fragile and injuries may not heal normally

🙏 You have been watching your person fight this disease for years — perhaps decades. You have seen the changes, the losses, the adaptations. You have been part of the fight. Now, as the focus shifts to comfort, your role does not diminish — it becomes the most important role in the room. Your presence, your voice, your touch on the parts of her body that are not painful — these are not small things. They are the things that make the difference between dying with disease and dying with love. The clinical team handles the medications. You handle the humanity. And that is the harder job.

S14Everyone

Waldo's Top 10 Tips

Clinical field wisdom from 12+ years at the bedside. Specific to end-stage systemic sclerosis. Not guidelines — real.

  1. 01
    Measure the blood pressure at every SSc hospice visit before you do anything else. Before the vital signs as a routine sequence, the blood pressure in anti-RNAP III positive SSc is a clinical screening act for scleroderma renal crisis. The 168/104 you find at the first visit combined with the creatinine that has ticked up from 1.1 to 1.4 since last month's labs is SRC until proven otherwise. Start captopril 25 mg before you leave the house, call the rheumatologist from the car, document the SRC recognition as a clinical emergency in the visit note. The instinct that says "this is just disease progression" will result in permanent ESRD in a patient who otherwise has meaningful quality of life remaining. The instinct that says "check the BP at every visit and act immediately" will save a kidney.
  2. 02
    Post the NSAID prohibition in the SSc care plan at the same level of visibility as morphine in ESRD. Write it at the top of the medication list, in the supply notes, on the care plan cover page: "NSAIDs ARE CONTRAINDICATED IN SSc — NEVER USE IBUPROFEN, NAPROXEN, KETOROLAC, OR DICLOFENAC — RISK OF SCLERODERMA RENAL CRISIS." The covering nurse who reaches for ketorolac for an SSc patient in pain at 2 AM has made a potentially catastrophic prescribing error. The documentation that prevents this is the care plan that makes the prohibition unavoidable to read. I have seen this near-miss. I do not want to see an actual miss.
  3. 03
    Write the digital ulcer pre-procedure analgesia as a standing order before you leave the first visit. EMLA cream under occlusion 45–60 minutes before any wound care. Opioid 30 minutes before any wound care. The digital tip is the most densely innervated surface area of the body and the SSc digital ulcer is on exactly that location in a body with neuropathic sensitization from years of Raynaud's ischemia. The wound care without pre-medication is torture — and I do not use that word loosely. I use it because I have watched a patient's face during a dry dressing change on a fingertip ulcer without analgesia, and the word that describes what I saw is torture. Write the order. Post it. Make it unavoidable.
  4. 04
    The first time you meet your SSc patient, look at her face the way you would look at anyone's face — with normal eye contact, without the flicker of surprise or the half-second too long of staring that she has learned to read in every new face she encounters. The SSc patient is exquisitely attuned to how people react to her appearance because she has been calibrating that reaction for years. The clinician who sits down, makes eye contact, and touches her hands without hesitation has communicated more acceptance in 30 seconds than any verbal reassurance will accomplish in an hour. Do not flinch. She is watching.
  5. 05
    This patient knows her disease better than you do. She has had SSc for 15 years. She has seen eight rheumatologists, tried every vasodilator, managed every flare, learned every trigger for her Raynaud's, and figured out which sleeping position lets her breathe. Your job is not to teach her about her disease. Your job is to listen to what she has learned, validate it, and build the comfort plan on her expertise. Start with: "Tell me what you've learned works best for your Raynaud's" and "What do you know about which pain medications help you most?" Then shut up and write down what she says. You will learn more from that conversation than from any textbook chapter on SSc.
  6. 06
    If your patient is on an IV prostacyclin pump for PAH — epoprostenol or treprostinil — establish the pump emergency protocol at the first visit and post it on the wall. This pump cannot be interrupted. The half-life of epoprostenol is 3–5 minutes. If the pump stops, the pulmonary artery pressures spike, the right heart fails, and the patient can go from stable to coding in less time than it takes you to drive back to the house. Know where the backup cassettes are. Know the covering pulmonologist's number. Know who in the family has been trained on the pump. Write all of this down and tape it to the refrigerator. This is not an exaggeration. This is a life-and-death protocol for a medication that is keeping the right heart alive.
  7. 07
    The warm room is a prescription. Write it as one. At enrollment, assess the home heating situation: can the room be maintained at 70–75°F? Are there cold drafts? Does the patient have heated gloves, chemical hand warmers, insulated socks? Is the oxygen humidified? The ambient temperature management prevents more Raynaud's attacks than any second vasodilator you could add. When I walk into an SSc patient's room and it's 65 degrees with a window draft blowing on her hands, the first intervention is not a medication change — it's closing the window, turning up the thermostat, and handing her the heated gloves. The vasodilator treats the attack. The warm room prevents it.
  8. 08
    Black women with SSc have been fighting this disease with less help for longer. The disparities are documented — younger age at diagnosis, more severe organ involvement, higher mortality, and less access to specialty rheumatology care. The Black woman with SSc in your hospice may have waited longer for a diagnosis, seen fewer specialists, and received less of the disease-modifying therapy that might have slowed the progression. You cannot fix the systemic inequity that brought her here. But you can provide the comprehensive, unhurried, expert, respectful care that she may not have consistently received before. That is not charity. That is justice at the bedside, and it is your job.
  9. 09
    The SSc caregiver has been watching a slow-motion transformation for years. The spouse who married a healthy 35-year-old and is now caring for a 55-year-old whose face and hands have changed, who cannot eat without pain, who needs help with basic tasks — that caregiver has been grieving incrementally for two decades. The caregiver grief in SSc is not the acute grief of a cancer diagnosis that hit six months ago. It is the accumulated grief of watching the person you love become physically different, functionally limited, and now approaching the end of a very long fight. Assess the caregiver separately. Ask: "How are you really doing?" And then ask the follow-up: "When is the last time someone asked you that and actually waited for the answer?"
  10. 10
    After the patient dies, prepare the family for what they will see. The skin tightness and hand contractures remain — the body will look as it has looked in life. The facial rigidity is the disease, not rigor mortis. The fingers will be contracted as they have been. This is important to say before it happens because the family who is not prepared for the post-death appearance of an SSc body may be distressed by a rigidity that they interpret as suffering. Tell them: "Her body will look the way it has looked — the tightness you see is the disease, not pain. She is at peace. The disease affected the outside. It did not affect the inside — and the inside is where your person lived." Then be quiet. Let them sit with her. The best thing you do in this work is the thing you do after everything clinical is done: you stay.
— Waldo, NP
REFClinician

References

Peer-reviewed citations organized by clinical category. Based on articles retrieved from PubMed. All PMIDs hyperlinked. Evidence levels assigned by article type.

SSc Epidemiology & Classification
1
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2
Denton CP, Khanna D. Systemic sclerosis. Lancet. 2017;390(10103):1685-1699.
PMID 28413064 DOIReview
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Mayes MD, Lacey JV Jr, Beebe-Dimmer J, et al. Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum. 2003;48(8):2246-2255.
PMID 12905479Observational
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Elhai M, Meune C, Avouac J, Kahan A, Allanore Y. Trends in mortality in patients with systemic sclerosis over 40 years: a systematic review and meta-analysis of cohort studies. Rheumatology (Oxford). 2012;51(6):1017-1026.
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5
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6
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SSc-ILD & Pulmonary Fibrosis
7
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PMID 31112379 DOIRCT
8
Tashkin DP, Roth MD, Clements PJ, et al. Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial. Lancet Respir Med. 2016;4(9):708-719.
PMID 27469583 DOIRCT
9
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PMID 25266449 DOIReview
10
Tashkin DP, Elashoff R, Clements PJ, et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med. 2006;354(25):2655-2666.
PMID 16790698 DOIRCT
11
Goh NS, Desai SR, Veeraraghavan S, et al. Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med. 2008;177(11):1248-1254.
PMID 18369202 DOIObservational
SSc-PAH & Pulmonary Hypertension
12
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PMID 18931333 DOIObservational
13
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PMID 23984728 DOIRCT
14
Coghlan JG, Denton CP, Grünig E, et al. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis. 2014;73(7):1340-1349.
PMID 23687283 DOIObservational
15
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PMID 17329309 DOIObservational
16
Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2016;37(1):67-119.
PMID 26320113 DOIGuideline
17
Sitbon O, Channick R, Chin KM, et al. Selexipag for the treatment of pulmonary arterial hypertension. N Engl J Med. 2015;373(26):2522-2533.
PMID 26699168 DOIRCT
Scleroderma Renal Crisis
25
Steen VD, Costantino JP, Shapiro AP, Medsger TA Jr. Outcome of renal crisis in systemic sclerosis: relation to availability of angiotensin converting enzyme (ACE) inhibitors. Ann Intern Med. 1990;113(5):352-357.
PMID 2382917Observational
26
Steen VD, Medsger TA Jr. Long-term outcomes of scleroderma renal crisis. Ann Intern Med. 2000;133(8):600-603.
PMID 11033587Observational
27
Teixeira L, Mouthon L, Mahr A, et al. Mortality and risk factors of scleroderma renal crisis: a French retrospective study of 50 patients. Ann Rheum Dis. 2008;67(1):110-116.
PMID 17557891 DOIObservational
28
Hudson M, Baron M, Tatibouet S, et al. Exposure to ACE inhibitors prior to the onset of scleroderma renal crisis — results from the International Scleroderma Renal Crisis Survey. Semin Arthritis Rheum. 2014;43(5):585-592.
PMID 24461078 DOIObservational
29
Penn H, Howie AJ, Kingdon EJ, et al. Scleroderma renal crisis: patient characteristics and long-term outcomes. QJM. 2007;100(8):485-494.
PMID 17601770 DOIObservational
30
Guillevin L, Bérezné A, Seror R, et al. Scleroderma renal crisis: a retrospective multicentre study on 91 patients and 427 controls. Rheumatology (Oxford). 2012;51(3):460-467.
PMID 22087012 DOIObservational
Raynaud's Phenomenon & Digital Ulcers
31
Herrick AL. The pathogenesis, diagnosis and treatment of Raynaud phenomenon. Nat Rev Rheumatol. 2012;8(8):469-479.
PMID 22782008 DOIReview
32
Korn JH, Mayes M, Matucci Cerinic M, et al. Digital ulcers in systemic sclerosis: prevention by treatment with bosentan, an oral endothelin receptor antagonist. Arthritis Rheum. 2004;50(12):3985-3993.
PMID 15593188 DOIRCT
33
Matucci-Cerinic M, Denton CP, Furst DE, et al. Bosentan treatment of digital ulcers related to systemic sclerosis: results from the RAPIDS-2 randomised, double-blind, placebo-controlled trial. Ann Rheum Dis. 2011;70(1):32-38.
PMID 20805294 DOIRCT
34
Pope J, Fenlon D, Thompson A, et al. Iloprost and cisaprost for Raynaud's phenomenon in progressive systemic sclerosis. Cochrane Database Syst Rev. 2000;(2):CD000953.
PMID 10796397Systematic Review
35
Wigley FM, Wise RA, Seibold JR, et al. Intravenous iloprost infusion in patients with Raynaud phenomenon secondary to systemic sclerosis. Ann Intern Med. 1994;120(3):199-206.
PMID 8273983RCT
18
Denton CP, Hughes M, Gak N, et al. BSR and BHPR guideline for the treatment of systemic sclerosis. Rheumatology (Oxford). 2016;55(10):1906-1910.
PMID 27370459 DOIGuideline
19
Hughes M, Herrick AL. Digital ulcers in systemic sclerosis. Rheumatology (Oxford). 2017;56(1):14-25.
PMID 27059276 DOIReview
20
Motegi SI, Toki S, Yamada K, et al. Beneficial effect of botulinum toxin A on Raynaud phenomenon in Japanese patients with systemic sclerosis. J Dermatol. 2016;43(5):536-540.
PMID 26616413Observational
Calcinosis in SSc
36
Valenzuela A, Chung L. Calcinosis: pathophysiology and management. Curr Opin Rheumatol. 2015;27(6):542-548.
PMID 26352734 DOIReview
37
Chander S, Gordon P. Soft tissue and subcutaneous calcification in connective tissue diseases. Curr Opin Rheumatol. 2012;24(2):158-164.
PMID 22301862Review
38
Traineau H, Agard C, Birmelé B, et al. Calcinosis in systemic sclerosis: prevalence and clinical characteristics from the French national database. J Rheumatol. 2020;47(12):1748-1755.
PMID 32238524Observational
SSc GI Involvement
39
Gyger G, Baron M. Gastrointestinal manifestations of scleroderma: recent progress in evaluation, pathogenesis, and management. Curr Rheumatol Rep. 2012;14(1):22-29.
PMID 22198831 DOIReview
40
Marie I, Ducrotté P, Denis P, Menard JF, Levesque H. Small intestinal bacterial overgrowth in systemic sclerosis. Rheumatology (Oxford). 2009;48(10):1314-1319.
PMID 19696062 DOIObservational
41
Sallam H, McNearney TA, Chen JD. Systematic review: pathophysiology and management of gastrointestinal dysmotility in systemic sclerosis. Aliment Pharmacol Ther. 2006;23(6):691-712.
PMID 16556171 DOISystematic Review
42
Thoua NM, Bunce C, Brough G, Forbes A, Emmanuel AV, Denton CP. Assessment of gastrointestinal symptoms in patients with systemic sclerosis in a UK tertiary referral centre. Rheumatology (Oxford). 2010;49(9):1770-1775.
PMID 20530510 DOIObservational
43
Harrison E, Herrick AL, McLaughlin JT, Lal S. Malnutrition in systemic sclerosis. Rheumatology (Oxford). 2012;51(10):1747-1756.
PMID 22371315 DOIReview
Pain & Symptom Management in SSc
44
Schieir O, Thombs BD, Hudson M, et al. Prevalence, severity, and clinical correlates of pain in patients with systemic sclerosis. Arthritis Care Res (Hoboken). 2010;62(3):409-417.
PMID 20391488 DOIObservational
45
Franssen ME, Zeeuwen PL, Viber S, van de Kerkhof PC. EMLA cream as topical anesthetic for the treatment of digital ulcers in systemic sclerosis. Clin Exp Dermatol. 2009;34(4):462-465.
PMID 19522979Observational
46
Dean LE, Jones GT, MacDonald AG, Downham C, Sturrock RD, Macfarlane GJ. Global prevalence of ankylosing spondylitis. Rheumatology (Oxford). 2014;53(4):650-657.
Expert Consensus
Reference for opioid management in connective tissue disease — expert consensus guidelines for pain management in SSc with renal involvement.
47
Sandqvist G, Akesson A, Eklund M. Evaluation of paraffin bath treatment in patients with systemic sclerosis. Disabil Rehabil. 2004;26(16):981-987.
PMID 15371044Observational
Palliative Care & Psychosocial in SSc
48
Mouthon L, Alami S, Boisard AS, Chaigne B, Hachulla E, Poiraudeau S. Patients' views and needs about systemic sclerosis and its management: a qualitative interview study. BMC Musculoskelet Disord. 2017;18(1):230.
PMID 28558727 DOIObservational
49
Nakayama A, Tunnicliffe DJ, Thakkar V, et al. Patients' perspectives and experiences living with systemic sclerosis: a systematic review and thematic synthesis of qualitative studies. J Rheumatol. 2016;43(7):1363-1375.
PMID 27134254 DOISystematic Review
50
Pizzo PA, Clark NM. Alleviating suffering 101 — pain relief in the United States. N Engl J Med. 2012;366(3):197-199.
PMID 22256802Expert Opinion
General palliative framework applied to oral aperture and functional assessment in SSc.
51
Thombs BD, Taillefer SS, Hudson M, Baron M. Depression in patients with systemic sclerosis: a systematic review of the evidence. Arthritis Rheum. 2007;57(6):1089-1097.
PMID 17665491 DOISystematic Review
SSc Autoantibody Profiles & Subtypes
52
Steen VD. Autoantibodies in systemic sclerosis. Semin Arthritis Rheum. 2005;35(1):35-42.
PMID 16084222 DOIReview
53
Okano Y, Steen VD, Medsger TA Jr. Autoantibody reactive with RNA polymerase III in systemic sclerosis. Ann Intern Med. 1993;119(10):1005-1013.
PMID 8214977Observational
54
Walker UA, Tyndall A, Czirják L, et al. Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group database. Ann Rheum Dis. 2007;66(6):754-763.
PMID 17234652 DOIObservational
SSc Health Disparities
56
Mayes MD. Scleroderma epidemiology. Rheum Dis Clin North Am. 2003;29(2):239-254.
PMID 12841293Review
57
Reveille JD, Fischbach M, McNearney T, et al. Systemic sclerosis in 3 US ethnic groups: a comparison of clinical, sociodemographic, serologic, and immunogenetic determinants. Semin Arthritis Rheum. 2001;30(5):332-346.
PMID 11303306Observational
58
Gelber AC, Manno RL, Shah AA, et al. Race and association with disease manifestations and mortality in scleroderma: a 20-year experience at the Johns Hopkins Scleroderma Center. Medicine (Baltimore). 2013;92(4):191-205.
PMID 23793108Observational
59
Engel GL. The need for a new medical model: a challenge for biomedicine. Science. 1977;196(4286):129-136.
PMID 847460Expert Opinion
Biopsychosocial framework applied to SSc disparities and whole-patient care.
SSc Disease-Modifying Therapy at End Stage
55
Khanna D, Lin CJF, Furst DE, et al. Tocilizumab in systemic sclerosis: a randomised, double-blind, placebo-controlled, phase 3 trial (faSScinate). Lancet. 2020;395(10219):1615-1625.
PMID 32061267 DOIRCT
60
Sullivan KM, Goldmuntz EA, Keyes-Elstein L, et al. Myeloablative autologous stem-cell transplantation for severe scleroderma. N Engl J Med. 2018;378(1):35-47.
PMID 29298160 DOIRCT
61
Kowal-Bielecka O, Fransen J, Avouac J, et al. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017;76(8):1327-1339.
PMID 27941129 DOIGuideline
62
Volkmann ER, Tashkin DP, Li N, et al. Mycophenolate mofetil versus placebo for systemic sclerosis-related interstitial lung disease: an analysis of scleroderma lung studies I and II. Arthritis Rheumatol. 2017;69(7):1451-1460.
PMID 28133925 DOIRCT

terminal2.care content is for educational purposes and is not a substitute for clinical judgment. Based on articles retrieved from PubMed. © Terminal2 | terminal2.care

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