title: [The importance of early diagnosis of Wegener's granulomatosis: a typical problem case for the otolaryngologist (author's transl)].
abstract: Although the etiology and pathogenesis of Wegener's granulomatosis is still obscure, the pathological process is assumed to belong to the group of immunologic diseases. Destructive lesions commonly appear in the midface, particularly in the nose, and are seen microscopically as necrotizing vasculitis. We report a 19 year old woman with undetected disease who was finally diagnosed by repeated biopsies through collaboration between an otolaryngologist and a pathologist. This emphasizes the importance of an early diagnosis for therapeutic reasons since cooperation with an internist prevented the patient from developing gross mid-facial lesions. Treatment involved corticosteroids, immune suppression, and temporary low-dose heparin. During a seven-year period of follow-up, limited tissue changes occurred. Wegener's granulomatosis without treatment will proceed to death, while early recognition and proper treatment can induce long-lasting remissions. Thus, it becomes necessary for head and neck clinicians to become familiar with symptoms of the initial stage of the disease since they must help to secure a proper diagnosis (particularly through biopsies when indicated).
mesh_terms: D000328:Adult; D001327:Autoimmune Diseases; D001379:Azathioprine / Q000627:therapeutic use; D005260:Female; D005448:Fluocortolone / Q000627:therapeutic use; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D008775:Methylprednisolone / Q000627:therapeutic use; D013995:Time
chemical_list: D005448:Fluocortolone; D001379:Azathioprine; D008775:Methylprednisolone
vernacular_title: Bedeutung der Frühdiagnose der Wegenerschen Granulomatose. Ein typischer Problemfall des HNO-Arztes.