| Limited cutaneous systemic sclerosis |
- Raynaud phenomenon for years, occasionally decades
- Skin involvement limited to hands, face, feet, and forearms (acral distribution)
- Nailfold capillary pattern typical of systemic sclerosis (predominantly nailfold capillary loops with capillary dropout)
- A significant (10 to 15%) late incidence of pulmonary hypertension, with or without skin calcification, gastrointestinal disease, telangiectasias (CREST syndrome), or interstitial lung disease
- Renal disease rarely occurs
- Anticentromere antibody (ACA) in 50 to 60%, but other patterns also occurring in 5 to 10% (especially anti-PM/Scl and anti-Scl-70)
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| Diffuse cutaneous systemic sclerosis |
- Raynaud phenomenon followed, within 1 year, by puffy or hidebound skin changes
- Truncal and acral skin involvement; tendon friction rubs
- Nailfold capillary pattern typical of systemic sclerosis with dilatation (early), dilatation and dropout (active), and tortuosity with dropout (late)
- Early and significant incidence of kidney, interstitial lung, diffuse gastrointestinal, and myocardial disease
- Anti-Scl-70 (30%) and anti-RNA polymerase I, II, or III (12 to 15%) antibodies
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| Systemic sclerosis sine scleroderma |
- Presentation with pulmonary fibrosis or kidney, cardiac, or gastrointestinal disease
- No skin involvement
- Raynaud phenomenon may be present
- Antinuclear antibodies may be present (anti-Scl-70, ACA, or anti-RNA polymerase I, II, or III)
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| Overlap syndromes |
- Features of systemic sclerosis that coexist with those of another autoimmune rheumatic disease, such as systemic lupus erythematosus, rheumatoid arthritis, dermatomyositis, vasculitis, or Sjögren's disease
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