CREST syndrome: a variant of progressive systemic sclerosis, associated with interstitial pulmonary fibrosis and malignancy.

We have described a patient who had a clinical picture of CREST syndrome and pulmonary interstitial fibrosis, and in whom adenocarcinoma of the lung developed over a four-year period. Despite absence in the literature of the association of lung carcinoma in patients with CREST syndrome, our case is an example of pulmonary fibrosis complicated by lung cancer without any evidence of other risk factors. We believe this to be the first report of such an association. Clinicians, therefore, must be aware that pulmonary interstitial fibrosis in patients with the CREST syndrome may represent a risk for lung cancer.