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Objectives-To review the autoimmune and rheumatic manifestations of patients with malignancy. Methods-A Medline search of all published papers using keywords related to malignancies, autoimmunity, rheumatic diseases, and paraneoplastic syndromes.

Objective. To determine whether the frequency of cancer is increased among patients with systemic sclerosis (SSc).Methods. A retrospective chart review of 248 patients who were followed up prospectively was conducted.Results. Cancers developed in 18 patients (7.3%) during 2,001 patient-years at risk. The most frequent types were cancers of the lung (7 patients) and breast (5 patients). Older age at diagnosis of SSc was a significant risk factor for cancer. Lung cancer was associated with the presence of pulmonary fibrosis. The age-standardized incidence rate for all cancers (7.9/1,000) was 2.1 times the overall rate in the Ontario population (P < 0.0001).Conclusion. The frequency of cancer is increased in patients with SSc.Since the report of alveolar cell carcinoma occurring in patients with systemic sclerosis (SSc; scleroderma) (l), a number of studies have indicated possible associations between SSc and breast, gastro- intestinal, urogenital, and hematologic malignancies (2-5). Several cases of breast cancer were noted to occur in close temporal relationship to the onset of SSc, suggesting an etiopathologic association (2).Whether cancer occurs more frequently in patients with SSc than in the general population is uncertain. Previous reports have shown the frequency of cancer in these patients to be between 3% and 7% (3,5,6), but other studies have found either no malignancies or very low prevalence (6-8). Duncan and Winkelmann (5) found the frequency and types of cancer seen in their large series of scleroderma patients to be similar to that seen in the overall patient population at the Mayo Clinic (Rochester, MN). The results from most published reports, however, are difficult to interpret either because of small numbers of patients or because the studies lacked suitable control populations for reliable comparison. Roumm and Medsger (3) compared the occurrence of cancer in scleroderma patients from the Pittsburgh area with National Cancer Survey statistics and found a slight increase in frequency among the scleroderma patients after adjustment for age and sex.To help clarify this issue, we reviewed the health records of 248 patients with SSc who had been followed prospectively over a 1Cyear period, for the occurrence of cancer. The observed frequency was standardized for age and compared with the incidence of cancer in the province of Ontario. PATIENTS AND METHODSThe health records of 248 patients who attended the Scleroderma Clinic at the Wellesley Hospital between 1978 and 1992 and were being followed up as part of an ongoing