{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,25]],"date-time":"2026-06-25T14:35:59Z","timestamp":1782398159856,"version":"3.54.5"},"reference-count":9,"publisher":"BMJ","issue":"11","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2023,11,5]]},"published-print":{"date-parts":[[2023,11]]},"abstract":"<jats:p>IgG<jats:sub>4<\/jats:sub>-related disease (IgG<jats:sub>4<\/jats:sub>-RD) is an inflammatory condition characterised by infiltration of tissue by IgG<jats:sub>4<\/jats:sub>-positive plasma cells. This is the seventh reported case of IgG<jats:sub>4<\/jats:sub>-RD affecting the mastoid and informs clinicians in diagnosing patients affected by this rare condition.<\/jats:p>\n               <jats:p>A woman in her 20s presented with unilateral otalgia, hearing loss and vertigo. She deteriorated despite antibiotic therapy and cross-sectional imaging revealed a destructive extra-axial lesion of the mastoid cells. Biopsy confirmed a diagnosis of IgG<jats:sub>4<\/jats:sub>-RD. She was successfully treated with prednisolone and azathioprine.<\/jats:p>\n               <jats:p>Inflammatory conditions should be considered in patients with persistent middle ear symptoms after infection and malignancy are excluded. Delays in diagnosis can lead to irreversible mass effects and may occur as current diagnostic criteria exclude mastoid-specific features.<\/jats:p>\n               <jats:p>IgG<jats:sub>4<\/jats:sub>-RD remains a rare diagnosis. To avoid significant effects on a patients\u2019 quality of life, prompt multidisciplinary treatment is vital alongside development of diagnostic criteria specific to otolaryngology.<\/jats:p>","DOI":"10.1136\/bcr-2022-253930","type":"journal-article","created":{"date-parts":[[2023,11,17]],"date-time":"2023-11-17T20:45:46Z","timestamp":1700253946000},"page":"e253930","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":3,"title":["IgG4-related disease of the mastoid: a rare presentation of a novel diagnosis"],"prefix":"10.1136","volume":"16","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5135-3971","authenticated-orcid":false,"given":"Mohit","family":"Achanta","sequence":"first","affiliation":[{"name":"Department of Otolaryngology, Imperial College Healthcare NHS Trust, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hisham","family":"Jaber","sequence":"additional","affiliation":[{"name":"Department of Radiology, University of Wales Hospital, Cardiff, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adaku","family":"Onovo","sequence":"additional","affiliation":[{"name":"Department of Cellular Pathology, University of Wales Hospital, Cardiff, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ben","family":"Stew","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology, University of Wales Hospital, Cardiff, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2023,11,17]]},"reference":[{"key":"2025082704074310000_16.11.e253930.1","doi-asserted-by":"crossref","unstructured":"Lanzillotta M , Mancuso G , Della-Torre E . Advances in the diagnosis and management of Igg4 related disease. BMJ 2020;369. doi:10.1136\/bmj.m1067","DOI":"10.1136\/bmj.m1067"},{"key":"2025082704074310000_16.11.e253930.2","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2012.72"},{"key":"2025082704074310000_16.11.e253930.3","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2019-216561"},{"key":"2025082704074310000_16.11.e253930.4","doi-asserted-by":"crossref","unstructured":"Maughan EF , Michaels J , Miller B , et al . Primary immunoglobulin G4-related Laryngeal disease: a case series and review of literature. Clin Med Insights Case Rep 2020;13. doi:10.1177\/1179547620960197","DOI":"10.1177\/1179547620960197"},{"key":"2025082704074310000_16.11.e253930.5","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1177\/0003489416678009","article-title":"Diagnostic challenges in a case of Igg4-RD affecting the temporal bone","volume":"126","author":"Vuncannon","year":"2017","journal-title":"Ann Otol Rhinol Laryngol"},{"key":"2025082704074310000_16.11.e253930.6","doi-asserted-by":"crossref","unstructured":"Cler SJ , Sharifai N , Baker B , et al . Igg4-related disease of the skull and skull base\u2013a systematic review and report of two cases. World Neurosurgery 2021. doi:10.1016\/j.wneu.2021.03.054","DOI":"10.1055\/s-0041-1725474"},{"key":"2025082704074310000_16.11.e253930.7","doi-asserted-by":"crossref","first-page":"314","DOI":"10.1007\/s12105-016-0710-0","article-title":"Recurrent Mastoiditis mimics Igg4 related disease: a potential diagnostic pitfall","volume":"10","author":"Deshpande","year":"2016","journal-title":"Head Neck Pathol"},{"key":"2025082704074310000_16.11.e253930.8","doi-asserted-by":"crossref","first-page":"577717","DOI":"10.1016\/j.jneuroim.2021.577717","article-title":"Igg4-related Pachymeningitis and Mastoiditis, associated with cerebral venous thrombosis: a case report","volume":"360","author":"Nilles","year":"2021","journal-title":"J Neuroimmunol"},{"key":"2025082704074310000_16.11.e253930.9","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1016\/j.neurad.2021.01.006","article-title":"Imaging of Igg4-related disease in the head and neck: a systematic review case series, and pathophysiology update","volume":"48","author":"Behzadi","year":"2021","journal-title":"J Neuroradiol"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2022-253930","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T11:07:51Z","timestamp":1756292871000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2022-253930"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,11]]},"references-count":9,"journal-issue":{"issue":"11","published-online":{"date-parts":[[2023,11,17]]},"published-print":{"date-parts":[[2023,11]]}},"alternative-id":["10.1136\/bcr-2022-253930"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2022-253930","relation":{},"ISSN":["1757-790X"],"issn-type":[{"value":"1757-790X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,11]]}}}