CREST Syndrome: A Benign Entity?-Reply

In Reply.— We doubt that any form of systemic scleroderma is a benign entity. However, we agree in part with Dr Brown's comments. The classification of systemic scleroderma, as stated, is difficult and arbitrary. In accordance with current usage, we attempted to classify patients with systemic scleroderma into those with the "CREST syndrome," "diffuse" scleroderma, and "inflammatory" scleroderma. The distinction is often difficult, and standardized criteria do not exist. Perhaps the anticentromere antibody and other autoantibodies will aid in the classification of scleroderma, so that it will not be based solely on a clinical differentiation. The "true speckles" or anticentromere antibody, frequently found in patients with CREST syndrome, has not been associated with renal involvement. 1 Therefore, although there have been reports of primary biliary cirrhosis and pulmonary hypertension in some patients with the CREST syndrome, this scleroderma variant is still relatively benign. In a previous study of 727