Classification of systemic sclerosis | Medibuddy
26th February 2025

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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