- Cutaneous: edema followed by fibrosis of the skin (chiefly extremities, face, trunk); telangiectasis; calcinosis; Raynaud's phenomenon
- Arthralgias and/or arthritis
- GI: esophageal hypomotility; intestinal hypofunction, gastric antral vascular ectasia (GAVE)
- Pulmonary: interstitial lung disease, pulmonary arterial hypertension, alveolitis
- Cardiac: pericarditis, cardiomyopathy, conduction abnormalities
- Renal: hypertension; renal crisis/failure
Two distinct subsets can be identified:
- Diffuse cutaneous SSc: rapid development of symmetric skin thickening of proximal and distal extremity, face, and trunk. At high risk for development of visceral disease early in course.
- Limited cutaneous SSc: often have long-standing Raynaud's phenomenon before other features appear; skin involvement limited to fingers (sclerodactyly), extremity distal to elbows, and face; generally associated with better prognosis but can be associated with pulmonary arterial hypertension; a subset of limited SSc has features of CREST syndrome (calcinosis, Raynaud's, esophageal dysmotility, sclerodactyly, telangiectasias).