Background: To determine the prevalence of connective tissue disease in a cohort of individuals with silicosis, we reviewed the medical records and questionnaires from individuals reported from 1987 to 1995 to a state surveillance system for silicosis. Reporting of individuals with silicosis is required by state law. Cases were reported by hospitals, physicians, the state workers' compensation bureau, or from death certificates. Only individuals who met the criteria for silicosis developed by the National Institute for Occupational Safety and Health (NIOSH) were included in the analysis. Results: A questionnaire was completed for all 583 cases. Medical records were available for 463. There were 24 people with rheumatoid arthritis, one with scleroderma, and one with systemic lupus erythematosus. All were men. The prevalence of rheumatoid arthritis was 5.2% (relative risk (RR) 2.73, 95% confidence limit (CL) 1.75-4.06). The prevalence of scleroderma was 0.2% (RR 15.65, 95% CL 0.21-87.03) and the prevalence of systemic lupus erythematosus was 0.2% (RR 11.37, 95% CL 0.15-63.23). This is an approximately 2.5-15-fold increased risk for these connective tissue diseases compared to estimated prevalences in the general population. Individuals with silicosis and connective tissue disease did not differ from individuals with silicosis but without connective tissue disease by race, age, type of industry where exposed to silica, history of tuberculosis, whether or not they had applied for workers' compensation, and whether or not they had progressive massive fibrosis on chest x-ray. Conclusion: Although the association between scleroderma and silicosis has been more widely reported in the literature, the prevalence of rheumatoid arthritis was greater than the prevalence of scleroderma or systemic lupus erythematosus among a cohort of individuals with silicosis.