Objective. To investigate the association between primary systemic vasculitis (PSV) and environmental risk factors.Methods. Seventy-five PSV cases and 273 controls (220 nonvasculitis, 19 secondary vasculitis, and 34 asthma controls) were interviewed using a structured questionnaire. Factors investigated were social class, occupational and residential history, smoking, pets, allergies, vaccinations, medications, hepatitis, tuberculosis, and farm exposure in the year before symptom onset (index year). The Standard Occupational Classification 2000 and job-exposure matrices were used to assess occupational silica, solvent, and metal exposure. Stepwise multiple logistic regression was used to calculate the odds ratio (OR) and 95% confidence interval Conclusion. A significant association between farming and PSV has been identified for the first time. Results also support previously reported associations with silica, solvents, and allergy.The primary systemic vasculitides (PSV) are a group of relatively rare diseases of unknown etiology (1). A number of potential environmental risk factors have been reported. Systemic vasculitis has been associated with exposure to particulate silica (e.g., quartz, granite, sandstone, and grain dust) (2-5). There has been conflicting evidence regarding a link between occupational exposure to hydrocarbons (e.g., paints, glues) and Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA), and the association with glomerulonephritis is stronger (6-8). A case-control study carried out at the National Institutes of Health (NIH) revealed an association with exposure to fumes or particulates and pesticides in patients with WG compared with healthy or rheumatic disease controls but not respiratory disease