[Wegener's granulomatosis in the head and neck region].
Wegener's granulomatosis (WG) is defined as a granulomatous inflammation of the upper and lower respiratory tract and systemic vasculitis of small and medium sized vessels which is often accompanied by a necrotizing glomerulonephritis. The etiology of the disease is still unknown. In former times untreated WG usually ended deadly. Immunosuppressive therapy made WG a treatable disease with a chronically relapsing course. Therefore an early diagnosis of WG is of utmost importance. WG usually starts as a limited and localized organ manifestation in the upper respiratory tract and it generalizes, if untreated, with pulmonary and renal involvement. Symptoms in the head and neck region are observed in up to 95% of the patients with WG. Sinusitis, crusting of the nose, development of a saddle nose, middle and inner ear symptoms and subglottic stenosis are common manifestations. Due to the early and common manifestation of WG in the head and neck region the otorhinolaryngologist plays an important role for the early diagnosis and the fast initiation of immunosuppressive therapy but also during follow-up for activity assessment.
Gottschlich
S
S
Klinik für Hals-, Nasen- und Ohrenheilkunde, Kopf- und Halschirurgie, Universitätsklinikum Schleswig-Holstein, Campus Kiel. gottschlich@hno.uni-kiel.de
Ambrosch
P
P
Gross
W L
WL
Hellmich
B
B
D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D003937:Diagnosis, Differential; D004724:Endoscopy; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007681:Kidney Papillary Necrosis / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D008171:Lung Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D008297:Male; D008875:Middle Aged; D009297:Nasal Mucosa / Q000473:pathology; D010038:Otorhinolaryngologic Diseases / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D011379:Prognosis; D012008:Recurrence
D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents