title: Clinical aspects of granulomatosis with polyangiitis affecting the head and neck.
abstract: There are many controversies in head and neck granulomatosis with polyangiitis (HN-GPA). Diagnostic/therapeutic regimens vary due to limited knowledge about the special properties of HN-GPA. 28 patients were diagnosed with GPA accordingly. Anti-neutrophil-cytoplasmatic-antibody (ANCA), anti-peroxidase-antibody (anti-PR3) and biopsies were performed for all patients and set into clinical context. 14 patients had sinonasal symptoms. Otological (n = 8) and laryngeal (n = 2) symptoms were usually associated with complex disease activity. Pulmonary and/or renal impairment was present in 14 patients at the time of diagnosis and developed in a further nine patients within 1 year. 21 patients with systemic disease displayed elevated ANCA/anti-PR3. In contrast, those with persistent isolated HN manifestations (n = 6) lacked auto-antibodies. These patients underwent multiple biopsies to diagnose GPA. Interestingly, five patients without clinical HN manifestations but elevated auto-antibodies were identified by nasal "blind" biopsy. Clinical examination, auto-antibody testing, and histology are effective diagnostic tools in HN-GPA. Histological diagnosis remains the gold standard in patients with persistent isolated head and neck manifestations but missing auto-antibodies. Based on our findings, we suggest early and sufficient systemic therapy for all HN-GPA. Nasal mucosal "blind" biopsy should be performed in patients with elevated auto-antibodies but lacking clinical head and neck manifestations.
authors.affiliation: Department of Otorhinolaryngology Head and Neck Surgery, Hals-Nasen-Ohrenklinik und Poliklinik, Technische Universität München, Ismaningerstrasse 22, 81675, Munich, Germany, a.knopf@lrz.tum.de.
authors.forename: Andreas
authors.initials: A
authors.lastname: Knopf
authors.forename: Adam
authors.initials: A
authors.lastname: Chaker
authors.forename: Thomas
authors.initials: T
authors.lastname: Stark
authors.forename: Benedikt
authors.initials: B
authors.lastname: Hofauer
authors.forename: Tobias
authors.initials: T
authors.lastname: Lahmer
authors.forename: Klaus
authors.initials: K
authors.lastname: Thürmel
authors.forename: Murat
authors.initials: M
authors.lastname: Bas
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001706:Biopsy; D019468:Disease Management; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis* / Q000628:therapy; D006257:Head; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009333:Neck; D012189:Retrospective Studies; D055815:Young Adult