Familial associations between ANCA-associated vasculitis and other immune-mediated inflammatory diseases: a case control study.

Authors

Justin Chong Meng Chua, Kevan Roy Polkinghorne, Samar Ojaimi, Jessica Ryan, Arthur Richard Kitching

Published in

Rheumatology international. Volume 46. Issue 8. Jul 24, 2026. Epub Jul 24, 2026.

Abstract

This study assesses the relationship between ANCA-associated vasculitis and immune-mediated inflammatory diseases (IMIDs) in first-degree relatives. 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. 3.27-16.09). 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. 2.46-6.62). 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.

PMID:
42496929
Bibliographic data and abstract were imported from PubMed on 24 Jul 2026.

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