Wegener granulomatosis in children and young adults - A case study of ten patients

This retrospective study reports seven children and three young adults (aged 11-30 years) who suffered from Wegener granulomatosis. Nine represent consecutive patients admitted to the Division of Nephrology over a period of 23 years. All patients had respiratory tract symptoms and renal involvement on admission. In several patients infiltrates on chest X-ray developed within 2 weeks of onset of symptoms. All patients survived. The median observation period was 9 years (range 13 months to 23 years). One patient progressed to endstage renal disease. Nine patients initially received cyclophosphamide and steroids. After a median period of 9 months (range 6-31 months) the cyclophosphamide was replaced by azathioprine. Relapses occurred after a median of 28 months (range 4-120 months) in 80% of patients, in six of the eight patients causing a definite decrease in kidney function. We believe that early diagnosis and initiation of therapy reduce the extent of organ damage. Since relapses are frequent, these patients should be evaluated frequently.