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People with AAV were matched 1-to-1 with control participants with no IMID in a case control study. Participants were asked whether their first-degree relatives had IMID. The likelihood of a positive family history was compared between cases and controls using conditional logistic regression. Additionally, the risk of first-degree relatives of cases having IMID was explored using generalised estimating equations. 145 AAV cases were matched with 145 controls. A family history of IMID was reported by 46.2% of those with AAV and 16.6% of controls. After adjustment for participant age and number of known family members, AAV cases were seven-times more likely to have a positive family history compared to controls (OR 7.25, 95% C.I. 3.27\u201316.09). 12% of first-degree relatives of AAV participants had an IMID compared to 3.5% of first-degree relatives of control participants. First-degree relatives of AAV participants were four-times more likely to have an IMID compared to a relative of a control (OR 4.03, 95% C.I. 2.46\u20136.62). Female first-degree relatives had double the risk of having an IMID, compared to male first-degree relatives. A history of IMID in first-degree family members is associated with an increased chance of a person developing AAV. Conversely, the presence of AAV in a first-degree relative is associated with having an increased chance of having an IMID. This implies a shared familial susceptibility between AAV and these diseases.<\/jats:p>","DOI":"10.1007\/s00296-026-06259-1","type":"journal-article","created":{"date-parts":[[2026,7,24]],"date-time":"2026-07-24T05:49:42Z","timestamp":1784872182000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Familial associations between ANCA-associated vasculitis and other immune-mediated inflammatory diseases: a case control study"],"prefix":"10.1007","volume":"46","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2399-9078","authenticated-orcid":false,"given":"Justin Chong Meng","family":"Chua","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9851-002X","authenticated-orcid":false,"given":"Kevan Roy","family":"Polkinghorne","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2129-1866","authenticated-orcid":false,"given":"Samar","family":"Ojaimi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2007-6021","authenticated-orcid":false,"given":"Jessica","family":"Ryan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2713-2391","authenticated-orcid":false,"given":"Arthur Richard","family":"Kitching","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,7,24]]},"reference":[{"issue":"1","key":"6259_CR1","doi-asserted-by":"publisher","first-page":"71","DOI":"10.1038\/s41572-020-0204-y","volume":"6","author":"AR Kitching","year":"2020","unstructured":"Kitching AR, Anders HJ, Basu N, Brouwer E, Gordon J, Jayne DR, Kullman J, Lyons PA, Merkel PA, Savage COS, Specks U, Kain R (2020) ANCA-associated vasculitis. 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Cureus 12(5). https:\/\/doi.org\/10.7759\/cureus.8094","DOI":"10.7759\/cureus.8094"}],"container-title":["Rheumatology International"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00296-026-06259-1.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00296-026-06259-1","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00296-026-06259-1.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T03:37:29Z","timestamp":1786937849000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00296-026-06259-1"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,7,24]]},"references-count":44,"journal-issue":{"issue":"8","published-online":{"date-parts":[[2026,8]]}},"alternative-id":["6259"],"URL":"https:\/\/doi.org\/10.1007\/s00296-026-06259-1","relation":{},"ISSN":["1437-160X"],"issn-type":[{"value":"1437-160X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,7,24]]},"assertion":[{"value":"2 May 2026","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"11 July 2026","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"24 July 2026","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"Justin Chua, Kevan Polkinghorne and Samar Ojaimi declare that they have no conflict of interest. Jessica Ryan is the secretary of the Australian and New Zealand Vasculitis Society. Arthur Richard Kitching has received research grants to his institution from Aventis, CSL Limited and Visterra Inc and consulting fees to his institution from Protalix, Sitala and Patrys, he has received royalties from Up to Date, has received payment for educational activities from CSL Limited, and he is the chair of the Australian and New Zealand Vasculitis Society.","order":1,"name":"Ethics","label":"Conflict of interest","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"This study was performed in line with the principles of the Declaration of Helsinki and was approved by the Monash Health Human Research Ethics Committee (RES-23-0000-391\u00a0A) on 19th September 2023.","order":2,"name":"Ethics","label":"Ethical approval","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Consent was obtained from all participants by ticking the consent box on the online survey page.","order":3,"name":"Ethics","label":"Informed consent","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"We declare that artificial intelligence has not been used to write or revise this manuscript. We declare that external commercial editing support has not been used to write or revise this manuscript. No part of this report is copied or published elsewhere.","order":4,"name":"Ethics","label":"Writing of manuscript","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"This research was presented at the 22nd International Vasculitis Workshop in 2026 with an abstract published in the Zenodo Repository. The full citation of the workshop abstract publication is: Kitching, A. R., Hill, C., & Quincey, V. (2026). Proceedings of the 22nd International Vasculitis Workshop, Melbourne, Feb 22\u201325, 2026. Proceedings of the 22nd International Vasculitis Workshop, 2026, 1\u2013552.\n                      \n                      . This research was also presented at the Australian and New Zealand Society of Nephrology Congress in 2025, with an abstract published in Nephrology. The full citation of the congress abstract publication is: 2025 \u201cMini Orals.\u201d Nephrology 30, no. S3: 24\u201365.\n                      \n                      .","order":5,"name":"Ethics","label":"Presentation of this research","group":{"name":"EthicsHeading","label":"Declarations"}}],"article-number":"221"}}