| Limited cutaneous systemic sclerosis |
- Raynaud phenomenon is usually the presenting sign
- Skin involvement limited to hands, face, feet, and forearms (acral distribution)
- Typical scleroderma nailfold capillary pattern
- A significant incidence of skin calcification, gastrointestinal disease, telangiectasias (CREST syndrome), with or without pulmonary hypertension or interstitial lung disease
- ACA in 7 to 10%, but other patterns also occurring, especially anti-PM/Scl (20%) and anti-Scl-70 (30%)
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| Diffuse cutaneous systemic sclerosis |
- Raynaud phenomenon followed by puffy fingers or hidebound skin changes
- Truncal and acral skin involvement
- Nailfold capillary pattern typical of systemic sclerosis with dilatation (early), dilatation and dropout (active), and tortuosity with dropout (late)
- Significant incidence of interstitial lung, diffuse gastrointestinal, and myocardial disease
- Anti-Scl-70 (33%), anti-PM/SCL (14%), and ACA (3%)
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| Systemic sclerosis sine scleroderma |
- Presentation with Raynaud phenomenon or peripheral vascular equivalent plus arthritis or cardiac, pulmonary, kidney, or gastrointestinal disease
- No skin involvement
- Main features: high frequency of cardiac involvement, delay in diagnosis, and poor outcome
- Positive ANA; specific ANA may be present (anti-Scl-70, ACA, or anti-RNA polymerase III)
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| Overlap syndromes |
- Features of systemic sclerosis that coexist with those of another connective tissue disease, such as systemic lupus erythematosus, rheumatoid arthritis, or dermatomyositis
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