CREST syndrome: a benign entity?

To the Editor.— In the July 1983Archives, Tuffanelli et al 1 made the potentially erroneous statement that the CREST (calcinosis, Raynaud's phenomenon, esophageal involvement, sclerodactyly, and telangiectasia) syndrome is a benign, chronic form of scleroderma. In the past, the CREST syndrome variant has been thought to represent a more benign form of scleroderma, generally without life-threatening visceral involvement. 2,3 However, numerous reports in the literature have suggested that the CREST syndrome is not benign. During a 15-year period, Rowell 4 followed up 84 patients with scleroderma, 12 of whom had CREST syndrome. The patients with CREST syndrome were found to have the same degree of systemic involvement and the same prognosis as did the other patients with scleroderma. The CREST syndrome has been associated with pulmonary hypertension resulting from progressive pulmonary vascular obliteration that can, in the absence of substantial pulmonary fibrosis, lead to death. 5 Ungerer et al