{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,27]],"date-time":"2026-01-27T23:58:15Z","timestamp":1769558295321,"version":"3.49.0"},"reference-count":14,"publisher":"BMJ","issue":"9","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2024,8,22]]},"published-print":{"date-parts":[[2024,9]]},"abstract":"<jats:p>Granulomatosis polyangiitis (GPA) is an autoimmune condition causing inflammation of small blood vessels. It is a rare disorder that may affect various parts of the body. The diagnosis is often based on clinical examination, laboratory investigations and tissue biopsy. In about 10\u201320% of patients, the anti-neutrophilic cytoplasmic antibody (ANCA) can be negative, and histology maybe inconclusive, which can lead to diagnostic uncertainty. Failure to treat vasculitis can lead to morbidity and even mortality. We present a case report of a gentleman who was presented with an airway emergency with inflammation of the nasal cavity and subglottic involvement amounting to airway stenosis. His ANCA was negative and tissue biopsy from the subglottis was inconclusive. He underwent urgent dilatation of his airway, local therapies to the nose and was commenced on 10 cycles of cyclophosphamide. A follow-up of over 4 years has not shown any relapse of his disease clinically or biochemically. We discuss the clinical findings, diagnostic dilemma and multidisciplinary management of this life-threatening condition.<\/jats:p>","DOI":"10.1136\/bcr-2023-258391","type":"journal-article","created":{"date-parts":[[2024,9,29]],"date-time":"2024-09-29T01:45:17Z","timestamp":1727574317000},"page":"e258391","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":1,"title":["ANCA-negative granulomatosis polyangiitis: an airway emergency"],"prefix":"10.1136","volume":"17","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1679-6993","authenticated-orcid":false,"given":"Nita","family":"Rachel Rajan","sequence":"first","affiliation":[{"name":"Department of ENT, Salford Care Organisation, Northern Care Alliance NHS foundation Trust, Salford, UK"},{"name":"Department of ENT, Tameside and Glossop IC NHS Foundation trust, Ashton-under-Lyne, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nina","family":"Brown","sequence":"additional","affiliation":[{"name":"Department of Renal Medicine, Salford Care Organisation, Northern Care Alliance NHS foundation trust, Salford, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Edmond","family":"O'Riordan","sequence":"additional","affiliation":[{"name":"Department of Renal Medicine, Salford Care Organisation, Northern Care Alliance NHS foundation trust, Salford, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chaitanya","family":"Gadepalli","sequence":"additional","affiliation":[{"name":"Department of ENT, Salford Care Organisation, Northern Care Alliance NHS foundation Trust, Salford, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2024,9,28]]},"reference":[{"key":"2025082706585846000_17.9.e258391.1","doi-asserted-by":"publisher","DOI":"10.1007\/s10157-013-0869-6"},{"key":"2025082706585846000_17.9.e258391.2","doi-asserted-by":"crossref","DOI":"10.5041\/RMMJ.10263","article-title":"Cocaine-induced vasculitis","volume":"7","author":"Berman","year":"2016","journal-title":"Rambam Maimonides Med J"},{"key":"2025082706585846000_17.9.e258391.3","first-page":"12","article-title":"Drug-induced vasculitis: a clinical and pathological review","volume":"70","author":"Radi\u0107","year":"2012","journal-title":"Neth J Med"},{"key":"2025082706585846000_17.9.e258391.4","doi-asserted-by":"crossref","unstructured":"Padoan R , Campaniello D , Felicetti M , et al . Ear, nose, and throat in ANCA-associated vasculitis: a comprehensive review. VP 2021. doi:10.20517\/2574-1209.2021.41","DOI":"10.20517\/2574-1209.2021.41"},{"key":"2025082706585846000_17.9.e258391.5","doi-asserted-by":"crossref","first-page":"i14","DOI":"10.1093\/ndt\/gfv022","article-title":"Classification, epidemiology and clinical subgrouping of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis","volume":"30 Suppl 1","author":"Watts","year":"2015","journal-title":"Nephrol Dial Transplant"},{"key":"2025082706585846000_17.9.e258391.6","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2016-210942"},{"key":"2025082706585846000_17.9.e258391.7","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2009.119032"},{"key":"2025082706585846000_17.9.e258391.8","doi-asserted-by":"crossref","DOI":"10.1136\/bmj.m1070","article-title":"ANCA associated vasculitis","volume":"369","author":"Hunter","year":"2020","journal-title":"BMJ"},{"key":"2025082706585846000_17.9.e258391.9","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2016-209133"},{"key":"2025082706585846000_17.9.e258391.10","doi-asserted-by":"crossref","first-page":"60","DOI":"10.7861\/clinmedicine.17-1-60","article-title":"ANCA-associated vasculitis","volume":"17","author":"Yates","year":"2017","journal-title":"Clin Med (Lond)"},{"key":"2025082706585846000_17.9.e258391.11","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1038\/s41572-020-0204-y","article-title":"ANCA-associated vasculitis","volume":"6","author":"Kitching","year":"2020","journal-title":"Nat Rev Dis Primers"},{"key":"2025082706585846000_17.9.e258391.12","doi-asserted-by":"crossref","first-page":"1038","DOI":"10.1001\/jamaoto.2014.2430","article-title":"Long-term outcome of airway stenosis in granulomatosis with polyangiitis (Wegener Granulomatosis)","volume":"140","author":"Martinez Del Pero","year":"2014","journal-title":"JAMA Otolaryngol Head Neck Surg"},{"key":"2025082706585846000_17.9.e258391.13","doi-asserted-by":"crossref","DOI":"10.1136\/rmdopen-2021-002160","article-title":"Comparative study of granulomatosis with polyangiitis subsets according to ANCA status: data from the French Vasculitis Study Group Registry","volume":"8","author":"Pu\u00e9chal","year":"2022","journal-title":"RMD Open"},{"key":"2025082706585846000_17.9.e258391.14","first-page":"111","article-title":"Clinical and prognostic features of Korean patients with MPO-ANCA, PR3-ANCA and ANCA-negative vasculitis","volume":"35 Suppl 103","author":"Yoo","year":"2017","journal-title":"Clin Exp Rheumatol"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2023-258391","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T13:59:10Z","timestamp":1756303150000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2023-258391"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,9]]},"references-count":14,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2024,9,28]]},"published-print":{"date-parts":[[2024,9]]}},"alternative-id":["10.1136\/bcr-2023-258391"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2023-258391","relation":{},"ISSN":["1757-790X"],"issn-type":[{"value":"1757-790X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,9]]}}}