Version June 2024
| Diffuse cutaneous | Early (<3 years after onset) | Late (>3 years after onset) |
| Constitutional | Fatigue and weight loss | Minimal, weight gain typical |
| Vascular | Raynaud often relatively mild | Raynaud more severe, more telangiectasia |
| Cutaneous | Rapid progression involving arms, trunk, face | Stable or regression |
| Musculoskeletal | Prominent arthralgia, stiffness, myalgia, muscle weakness, tendon friction rubs | Flexion contractures and deformities, joint/muscle symptoms less prominent |
| Gastrointestinal | Dysphagia, heartburn | More pronounced symptoms, midgut and anorectal complications more common |
| Cardiopulmonary | Maximum risk for myocarditis, pericardial effusion, interstitial pulmonary fibrosis | Reduced risk of new involvement but progression of existing established visceral fibrosis |
| Renal | Maximum risk for renal crisis within the first 5 years | Renal crisis less frequent, uncommon after 5 years |
| Limited cutaneous | Early (<10 years after onset) | Late (>10 years after onset) |
| Constitutional | None | Only secondary to visceral complications |
| Vascular | Raynaud typically severe and longstanding telangiectasia | Raynaud persists, often causing digital ulceration or gangrene |
| Cutaneous | Mild sclerosis with little progression on trunk, face | Stable, calcinosis more prominent |
| Musculoskeletal | Occasional joint stiffness | Mild flexion contractures |
| Gastrointestinal | Dysphagia, heartburn | More pronounced symptoms, midgut and anorectal complications more common |
| Cardiopulmonary | Usually no involvement | Lung fibrosis may develop, but often progresses slowly, anti-SCL-70 predicts increased risk of severe fibrosis. Maximum risk for developing isolated pulmonary hypertension and secondary right ventricular failure. |
| Renal | No involvement | Rarely involved, anti-RNA polymerase predicts increased risk of renal involvement. |
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