Limited systemic sclerosis

Definition

Patients with limited cutaneous SSc (lcSSc) generally have long-standing Raynaud's phenomenon before other manifestations of SSc appear. Skin involvement in lcSSc is slowly progressive and remains limited to the fingers (sclerodactyly), distal extremities, and face, but the trunk is not affected. Pulmonary arterial hypertension, interstitial lung disease (less common compared to diffuse systemic sclerosis), hypothyroidism, and primary biliary cirrhosis may develop in the late stage of lcSSc. A combination of calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, and teleangiectasia in patients with limited systemic sclerosis is called CREST syndrome.

Index Terms