Co-occurrence between C1 esterase inhibitor deficiency and autoimmune disease: a systematic literature review

BackgroundHereditary angioedema (HAE) is caused by a SERPING1 gene defect resulting in decreased (Type I) or dysfunctional (Type II) C1 esterase inhibitor (C1-INH). The prevalence of autoimmune diseases (ADs) in patients with HAE appears to be higher than the general population. A systematic literature review was conducted to examine the co-occurrence between HAE and ADs.MethodsPubMed/EMBASE were searched for English-language reviews, case …