{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,20]],"date-time":"2025-10-20T10:22:07Z","timestamp":1760955727781,"version":"3.44.0"},"reference-count":22,"publisher":"BMJ","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Reports"],"accepted":{"date-parts":[[2017,6,21]]},"abstract":"<jats:p>Skull base osteomyelitis in the setting of granulomatosis with polyangiitis (GPA) is rare and entails significant diagnostic challenges. We present a case of a 65-year-old Caucasian man with a history of rheumatoid arthritis, off immunosuppression for 18 months, who presented with 2\u2009years of chronic headaches, severe fatigue, saddle nose deformity and 20-kilogram unintentional weight loss. Maxillofacial CT revealed an extensive destructive sinonasal and erosive skull base process. Laboratory evaluation showed equivocal elevation of antiproteinase 3 antibodies with negative antineutrophil cytoplasmic antibody panel. Biopsy of the skull base\/clivus revealed necrotising granulomatous inflammation with focal vasculitis consistent with GPA, and multiple bone cultures were positive for <jats:italic>Pseudomonas aeruginosa<\/jats:italic>. This patient was diagnosed concurrently with GPA and <jats:italic>P. aeruginosa<\/jats:italic> skull base osteomyelitis. He was started on a 6-week course of cefepime intravenously and oral prednisone, with the plan to initiate rituximab infusion 2\u2009weeks after initiation of antibiotic therapy.<\/jats:p>","DOI":"10.1136\/bcr-2017-220135","type":"journal-article","created":{"date-parts":[[2017,7,19]],"date-time":"2017-07-19T08:50:18Z","timestamp":1500454218000},"page":"bcr-2017-220135","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":5,"title":["Severe destructive nasopharyngeal granulomatosis with polyangiitis with superimposed skull base <i>Pseudomonas aeruginosa<\/i> osteomyelitis"],"prefix":"10.1136","volume":"2017","author":[{"given":"Mitchell S","family":"von Itzstein","sequence":"first","affiliation":[{"name":"School of Medicine, Griffith University, Gold Coast, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jithma P","family":"Abeykoon","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniel D","family":"Summerfield","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jennifer A","family":"Whitaker","sequence":"additional","affiliation":[{"name":"Division of Infectious Diseases, Mayo Clinic, Rochester, Minnesota, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2017,7,19]]},"reference":[{"key":"2025082802582085000_2017.jul19_1.bcr-2017-220135.1","first-page":"1310","article-title":"Central skull base osteomyelitis in patients without otitis externa: imaging findings","volume":"24","author":"Chang","year":"2003","journal-title":"Am J Neuroradiol"},{"key":"2025082802582085000_2017.jul19_1.bcr-2017-220135.2","doi-asserted-by":"crossref","first-page":"073","DOI":"10.1055\/s-0029-1214159","article-title":"Osteomyelitis of the craniofacial skeleton","volume":"23","author":"Pincus","year":"2009","journal-title":"Semin Plast Surg"},{"key":"2025082802582085000_2017.jul19_1.bcr-2017-220135.3","doi-asserted-by":"crossref","first-page":"8","DOI":"10.21053\/ceo.2016.9.1.8","article-title":"Granulomatosis with polyangiitis in otolaryngologist practice: a review of current knowledge","volume":"9","author":"Wojciechowska","year":"2016","journal-title":"Clin Exp Otorhinolaryngol"},{"key":"2025082802582085000_2017.jul19_1.bcr-2017-220135.4","first-page":"S236","article-title":"Selected lesions of the sinonasal tract and nasopharynx","volume":"54","author":"Barnes","year":"2004","journal-title":"Pathology Int"},{"key":"2025082802582085000_2017.jul19_1.bcr-2017-220135.5","first-page":"63","article-title":"Lessons learnt in the management of Wegener's Granulomatosis: long-term follow-up of 60 patients","volume":"45","author":"Sproson","year":"2007","journal-title":"Rhinology"},{"key":"2025082802582085000_2017.jul19_1.bcr-2017-220135.6","doi-asserted-by":"crossref","first-page":"1083","DOI":"10.1002\/lary.24440","article-title":"Central Skull base osteomyelitis: an emerging clinical entity","volume":"124","author":"Johnson","year":"2014","journal-title":"Laryngoscope"},{"key":"2025082802582085000_2017.jul19_1.bcr-2017-220135.7","doi-asserted-by":"publisher","DOI":"10.1017\/S0022215114001492"},{"key":"2025082802582085000_2017.jul19_1.bcr-2017-220135.8","doi-asserted-by":"crossref","first-page":"1101","DOI":"10.1002\/art.1780330807","article-title":"The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis","volume":"33","author":"Leavitt","year":"1990","journal-title":"Arthritis Rheum"},{"key":"2025082802582085000_2017.jul19_1.bcr-2017-220135.9","doi-asserted-by":"publisher","DOI":"10.1002\/art.37715"},{"key":"2025082802582085000_2017.jul19_1.bcr-2017-220135.10","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1213277"},{"key":"2025082802582085000_2017.jul19_1.bcr-2017-220135.11","doi-asserted-by":"crossref","DOI":"10.1097\/MD.0000000000000747","article-title":"Positron emission tomography scanning in anti-neutrophil cytoplasmic antibodies-associated vasculitis","volume":"94","author":"Kemna","year":"2015","journal-title":"Medicine"},{"key":"2025082802582085000_2017.jul19_1.bcr-2017-220135.12","doi-asserted-by":"publisher","DOI":"10.1080\/00016480600818054"},{"key":"2025082802582085000_2017.jul19_1.bcr-2017-220135.13","doi-asserted-by":"publisher","DOI":"10.1093\/cid\/cir842"},{"key":"2025082802582085000_2017.jul19_1.bcr-2017-220135.14","doi-asserted-by":"crossref","unstructured":"Harrison L , Mcnally J , Corbridge R . 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