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Scleroderma (Systemic Sclerosis)

Eva Niklinska and Raeann Whitney


  • Distinguish from localized scleroderma (morphea or linear scleroderma) = dermal fibrosis w/o 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

      • Limited does not equal mild, limited refers to distribution of skin involvement

      • Renal crisis is rare

      • Patients with + ACA (anticentromere Ab) are at high risk for developing PAH

      • Patients with + Anti-Scl-70 Ab are also at high risk for progressive ILD

    • Diffuse Cutaneous: skin thickening extends proximal to the elbows/knees or trunk

      • Typically more abrupt onset and rapid progression compared to lcSSc

      • 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)

  • Systemic: fatigue, weight loss

  • Vascular: Raynaud’s ± digital tip ulcers, telangiectasias, nailfold capillaroscopy w/ dilated capillary loops

  • Skin: 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 (PAH)

  • 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 weight-based symptom scoring  
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-cL 70], anti-RNA polymerase III) 3
Patients with a total score of ≥9 are classified as having definite systemic sclerosis (sensitivity 91%, specificity 92%)
  • Labs: ANA w/ Reflex, Scl70, anticentromere, RNA pol III (separate order in Epic)

  • 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)


  • Organ-Based Symptomatic Therapy

    • Raynauds: CCB (amlodipine, nifedipine), topical nitroglycerin

    • GERD: PPI

    • Renal: Monitor BP; Cr

    • ILD: Periodic PFTs, monitor for new respiratory symptoms, pulm referral

    • Cardiac/PAH: annual TTE, cardiology referral

  • Systemic Immunosuppression (if progressive skin thickening or organ involvement)

    • MTX, MMF, cyclophosphamide, if refractory rituximab, IVIG 

Scleroderma Renal Crisis

  • Typically presents early in disease course (can even precede skin thickening)

  • Abrupt onset of hypertension and renal dysfunction

  • Labs: Elevated Cr, proteinuria, microangiopathic hemolytic anemia, elevated renin

  • Treatment: short acting ACE (captopril or enalapril, titratable), may require HD

Last update: 2022-06-26 16:51:01