Richard M. Silver (editor) - Case Studies in Systemic Sclerosis

Richard M. Silver 1

(1)

Abstract

Systemic sclerosis (scleroderma) is classified based on the extent of skin involvement as either limited cutaneous or diffuse cutaneous scleroderma. Patients with the limited cutaneous form of systemic sclerosis, once known as the CREST syndrome (Calcinosis, Raynauds phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasias), often report a long history of Raynauds phenomenon preceding other clinical manifestations. Current classification criteria lack sensitivity for this subset of scleroderma, as it may take years or even decades before these patients fulfill classification criteria. Certain clinical and laboratory findings can facilitate earlier diagnosis, particularly the presence of digital pitted scars, telangiectasias, abnormal nailfold capillary morphology, and the presence of anticentromere antibodies (ACA). One should consider the diagnosis of limited cutaneous systemic sclerosis in any patient who gives a history of Raynauds phenomenon with one or more of these clinical and laboratory features, or with signs and symptoms suggesting visceral organ involvement. Although limited in the extent of skin involvement, these patients must be monitored for the development of internal organ complications such as pulmonary arterial hypertension, pulmonary fibrosis, and a variety of gastrointestinal complications.

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