| 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) |
| 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 renal, interstitial lung, diffuse gastrointestinal, and myocardial disease |
| Anti-Scl-70 (30%) and anti-RNA polymerase I, II, or III (12 to 15%) antibodies |
| Systemic sclerosis sine scleroderma |
| Presentation with pulmonary fibrosis or renal, 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) |
| 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 syndrome |
