title: Wegener granulomatosis in children and young adults. A case study of ten patients.
abstract: 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 end-stage 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.
authors.affiliation: Department of Internal Medicine, University Hospital, Umeå, Sweden. Bernd.Stegmayr@medicin.umu.se
authors.forename: B G
authors.initials: BG
authors.lastname: Stegmayr
authors.forename: L
authors.initials: L
authors.lastname: Gothefors
authors.forename: B
authors.initials: B
authors.lastname: Malmer
authors.forename: D E
authors.initials: DE
authors.lastname: Müller Wiefel
authors.forename: K
authors.initials: K
authors.lastname: Nilsson
authors.forename: B
authors.initials: B
authors.lastname: Sundelin
mesh_terms: D000293:Adolescent; D000328:Adult; D000937:Antigen-Antibody Reactions; D001379:Azathioprine / Q000627:therapeutic use; D002648:Child; D003520:Cyclophosphamide / Q000627:therapeutic use; D018450:Disease Progression; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000188:drug therapy / Q000453:epidemiology / Q000276:immunology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015994:Incidence; D007668:Kidney / Q000503:physiopathology; D007674:Kidney Diseases / Q000209:etiology; D008297:Male; D012008:Recurrence; D012140:Respiratory Tract Diseases / Q000209:etiology; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D001379:Azathioprine