Complement 1 esterase inhibitor (C1-INH) is a serine protease inhibitor, which is an important regulatory substance in the plasma cascade reaction and can regulate the complement system and bradykinin-forming system. hereditary angioedema (HAE) is a rare autosomal dominant inherited disease, mainly caused by a mutation of the gene controlling the synthesis of C1-INH resulting in reduced or dysfunctional levels of C1-INH in patients. C1-INH substitution therapy is one of the main therapeutic strategies for controlling HAE attacks. This article mainly collates and analyzes related research literature, summarizes the progress of C1-INH treatment of HAE, and provides some references for clinicians to treat HAE.
