A large case–control study comparing incident cases of granulomatosis with polyangiitis (GPA; n = 757) with population-based controls matched for age and sex (n = 7,546) found that a history of bronchiectasis up to 5 years before GPA diagnosis was strongly associated with GPA (OR 5.1; P < 0.0001). Moreoever, individuals with GPA were two to three times more likely than controls to have a previous diagnosis of an autoimmune disease or chronic renal impairment. Pulmonary fibrosis in the previous 3 years was also identified as a risk factor for GPA (OR 5.7; P = 0.01).