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

This retrospective study reports seven children and three young adults (age d 11-30 years) who suffered from Wegener granulomatosis. Nine represent con secutive patients admitted to the Division of Nephrology over a period of 2 3 years. All patients had respiratory tract symptoms and renal involvement on admission. In several patients infiltrates on chest X-ray developed with in 2 weeks of onset of symptoms. All patients survived. The median observat ion period was 9 years (range 13 months to 23 years). One patient progresse d to endstage renal disease. Nine patients initially received cyclophospham ide and steroids. After a median period of 9 months (range 6-31 months) the cyclophosphamide was replaced by azathioprine. Relapses occurred after a m edian of 28 months (range 4-120 months) in 80% of patients, in six of the e ight patients causing a definite decrease in kidney function. We believe th at early diagnosis and initiation of therapy reduce the extent of organ dam age. Since relapses are frequent, these patients should be evaluated freque ntly.