title: Clinical manifestations of granulomatosis with polyangiitis (Wegener's granulomatosis) in the upper respiratory tract seen by otolaryngologists in Japan.
abstract: We studied 91 patients with granulomatosis with polyangiitis (GPA; Wegener's granulomatosis) who were dealt with by otolaryngologists in Japan. The upper respiratory tract (URT) alone was involved in 56%. Regarding findings of PR3-ANCA, histology and initial diagnosis, of the 40 patients in whom sites other than the URT were involved, 64 and 73% were positive for PR3-ANCA and histology, respectively. On the other hand, of 51 patients in whom only the URT was involved, only 49 and 31% were positive for PR3-ANCA and histology, respectively. With regard to diagnosis, definitive and probable diagnoses were made in 78 and 20%, respectively, of patients involving sites other than the URT. On the other hand, definitive and probable diagnoses were made in only 22 and 37%, respectively, of patients involving the URT alone; the Japanese diagnostic criteria were inapplicable to 41%. Of the 21 patients to whom the diagnostic criteria were inapplicable, 13 (62%) developed additional symptoms and signs during the observation period, and they then fulfilled the Japanese diagnostic criteria. Among these patients, 8 were MPO-ANCA-positive and 3 patients had 2 or more sub-lesions in the URT. If the diagnostic criteria included cases with MPO-ANCA (±) and 2 lesions in the URT, the diagnostic rates increased from 59 to 86% even though they were of limited form. Thus, about 60% of the patients with URT symptoms alone did not satisfy the Japanese diagnostic criteria at the initial visit.
authors: Department of Otolaryngology-Head and Neck Surgery, Asahikawa Medical University, Midorigaoka-Higashi 2-1-1-1, Asahikawa, 078-8510, Japan. hyasu@asahikawa-med.ac.jp.
authors: Yasuaki
authors: Y
authors: Harabuchi
authors: Department of Otolaryngology-Head and Neck Surgery, Asahikawa Medical University, Midorigaoka-Higashi 2-1-1-1, Asahikawa, 078-8510, Japan.
authors: Kan
authors: K
authors: Kishibe
authors: Department of Otolaryngology-Head and Neck Surgery, Asahikawa Medical University, Midorigaoka-Higashi 2-1-1-1, Asahikawa, 078-8510, Japan.
authors: Yuki
authors: Y
authors: Komabayashi
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000032:analysis*; D015415:Biomarkers / Q000032:analysis; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D019538:Health Care Surveys; D006801:Humans; D007564:Japan; D008297:Male; D008875:Middle Aged; D053146:Myeloblastin / Q000276:immunology*; D009504:Neutrophils / Q000201:enzymology / Q000276:immunology*; D010036:Otolaryngology; D009195:Peroxidase / Q000276:immunology*; D011237:Predictive Value of Tests; D012137:Respiratory System / Q000276:immunology* / Q000473:pathology*; D012189:Retrospective Studies; D011795:Surveys and Questionnaires; D055815:Young Adult
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D015415:Biomarkers; D009195:Peroxidase; D053146:Myeloblastin