Classification of systemic sclerosis
| Limited cutaneous SSc | Diffuse cutaneous SSc | |
|---|---|---|
| Pattern of skin involvement | Only head and distal to elbow/knee | Diffuse skin involvement, including skin proximal to elbow/knee |
| Autoantibody | >95% ANA
70%-80% anti-centromere |
>95% ANA
60% Scl-70 (topoisomerase) Also U3RNP, RNA polymerase III and other rarer antibodies |
| Lung | PAH > pulmonary fibrosis | Pulmonary fibrosis |
| Scleroderma renal crisis | Lesser risk | Greater risk |
Table 4 Classification of systemic sclerosis and associated features
| Raynaud’s phenomenon
Raynaud’s phenomenon is classified as primary or secondary. Primary Raynaud’s generally affects young females early in life, with symmetrical involvement. There is not usually digital ulceration or nailfold vasculopathy. Secondary Raynaud’s is associated with rheumatological conditions such as systemic sclerosis, SLE and inflammatory myopathies, and haematological disorders such as cryoglobulinaemia and POEMS syndrome. Conservative measures such as gloves, hand warmers and smoking cessation are first-line. Pharmacological treatment can be used, such as nifedipine, losartan, sildenafil or fluoxetine. Combination therapies can be used if still symptomatic and blood pressure allows. |
Box 2 Raynaud’s phenomenon
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