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Scleroderma (Systemic Sclerosis)

Eva Niklinska and Raeann Whitney


  • Distinguish from localized scleroderma (morphea or linear scleroderma) which is dermal fibrosis without internal organ involvement
  • 2 Major Subtypes of Generalized / Systemic Scleroderma (SSc):
    • Limited cutaneous (lcSSc): Skin thickening limited to the neck, face, or distal to elbows and knees; spares the truck and proximal extremities. Raynaud’s may develop years before other manifestations, which then slowly accumulate over 5-10 years.
        • anticentromere antibody
        • Renal crisis is rare
      • High risk for developing PAH
    • Diffuse cutaneous: skin thickening extends proximal to the elbows/knees or trunk; rapid development of cutaneous and multiorgan involvement
      • More associated with anti-Scl-70 ab
      • Typically more abrupt onset and rapid progression compared to limited
      • High risk for progressive ILD
        • RNA polymerase III Ab = high risk of renal crisis, higher risk for malignancy


  • CREST: Calcinosis cutis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasia (CREST syndrome is no longer a discrete diagnosis but incorporated into lcSSc)
  • Systemic: fatigue, weight loss
  • Vascular: Raynaud’s ± digital tip ulcers, telangiectasias, nailfold capillaroscopy with dilated capillary loops
  • Skin: Sclerodactyly, loss of facial wrinkles, decreased oral aperture
  • MSK: arthralgias, myalgias, flexion contractures
  • GI: Esophageal or intestinal dysmotility, GERD, GAVE (watermelon stomach)
  • Pulm: ILD (NSIP, UIP), pulmonary arterial hypertension
  • Cardiac: pericardial effusions, myocarditis, cardiomyopathy, conduction system disease
  • Renal: renal crisis (onset of malignant HTN, greatest risk in first 5 years)


  • 2013 ACR/EULAR Classification Criteria à weight-based symptom scoring
  • Labs: ANA w/ Reflex, Scl70, anticentromere, RNA pol III (separate order in Epic, increased risk of scleroderma renal disease)
  • Imaging/Procedures: Baseline PFTs, lung HRCT, TTE, EKG, 6-minute walk test
  • Skin biopsy: Not often used for dx, may be required to differentiate other rare disorders (eosinophilic fasciitis, scleroderma, scleromyxedema)
2013 ACR/EULAR Classification Criteria Items Sub-items Weight
Skin thickening of fingers of both hands extending proximal to metacarpophalangeal (MCP) joints 9
Skin thickening of fingers (only count the highest score) Puffy fingers
Whole finger, distal to MCP
Fingertip lesions (only count the highest score) Digital tip ulcers
Pitting scars
Telangiectasia 2
Abnormal nailfold capillaries 2
Pulmonary arterial hypertension and/or interstitial lung disease 2
Raynaud’s phenomenon 3
Scleroderma-related antibodies (any of anti-centromere, anti-topoisomerase I [anti-ScL 70], anti-RNA polymerase III) 3
Pts with a total score of ≥9 are classified as having definite systemic sclerosis (sensitivity 91%, specificity 92%)


  • Organ-Based Symptomatic Therapy
    • Raynauds: CCB (amlodipine, nifedipine), topical nitroglycerin
    • GERD: PPI
    • Renal: Monitor BP and Cr
  • Scleroderma renal crisis: Abrupt onset of HTN and renal dysfunction that typically presents early in disease course (can precede skin thickening)
    • Labs: Elevated Cr, proteinuria, MAHA, elevated renin
    • Treatment: Short acting ACEi (captopril, enalapril), may require HD
  • ILD: Periodic PFTs (isolated DLCO may suggest PAH); monitor for respiratory symptoms, pulmonology referral
  • Cardiac/PAH: annual TTE, cardiology referral
  • Systemic Immunosuppression (if progressive skin thickening or organ involvement)
    • MTX, MMF, cyclophosphamide, if refractory rituximab, IVIG
    • Nintedanib may be used in combination with immunosuppression