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By Mayo Clinic staffThe cause of Wegener's granulomatosis is unknown, but it appears to develop as a result of an initial inflammation-causing event that triggers an abnormal reaction from your immune system. The combination of these events can lead to inflamed, constricted blood vessels and harmful inflammatory tissue masses (granulomas).
Scientists speculate that the initial event may be an infection. This has been documented in nearly half the people with Wegener's, but the infections aren't overwhelming. Other possible contributing causes include an environmental toxin, a genetic predisposition to the condition or even a combination of all of these. This question has yet to be resolved.