Type of disease | Typical features |
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Limited systemic sclerosis (previously called the CREST* syndrome) | - Skin lesions distal to the knees and elbows
- Raynaud's phenomenon occurring a long time before the other symptoms
- Commonly late complications
- Commonly elevated pulmonary arterial pressure and severe bowel disease
- Centromere antibodies
|
Diffuse (generalized) systemic sclerosis | - Skin lesions more proximally and also on the trunk
- Raynaud's phenomenon either concomitantly with or slightly before the other symptoms
- Increased risk of renal lesions, and heart symptoms
- Severe interstitial lung disease (ILD)
- Type I topoisomerase (Scl-70) antibodies, RNA polymerase III antibodies
|
Systemic sclerosis in the absence of scleroderma | - No thickening of the skin
- Raynaud's phenomenon
- Internal organ lesions or circulatory changes
- Typical serological findings associated with systemic sclerosis or capillaroscopic changes consistent with systemic sclerosis
|