Lack of increased prevalence of immunoregulatory disorders in hereditary angioedema due to C1-inhibitor deficiency

Hereditary angioedema due to deficiency of C1-INH (HAE-C1-INH) is associated with enhanced consumption of the early complement components, which may predispose for autoimmune disease.We assessed the prevalence of such disorders among HAE- C1-INH patients and their impact on the natural course of HAE-C1-INH.Clinical data and immunoserological parameters of 130 HAE-C1-INH and 174 non-C1-INH-deficient patients with angioedema were analyzed.In our study, the incidence of immunoregulatory disorders was 11.5% in the population of HAE-C1-INH patients and 5.2% among non-C1-INH-deficient controls with angioedema. Immunoserology screening revealed a greater prevalence of anticardiolipin IgM (p = 0.0118) among HAE-C1-INH patients, than in those with non-C1-INH-deficient angioedema.We did not find higher prevalence of immunoregulatory disorders among our HAE-C1-INH patients. However, in patients with confirmed immunoregulatory disorders, the latter influenced both the severity of HAE-C1-INH and the effectiveness of its long-term management. Appropriate management of the immunoregulatory disease thus identified improves the symptoms of HAE-C1-INH.

► Incidence of immunoregulatory disorders was 11.5% among our HAE-C1-INH patients. ► We did not find higher prevalence of these diseases among our HAE-C1-INH patients. ► Immunoregulatory disorders may influence severity of HAE and therapeutic efficacy. ► Management of immunoregulatory diseases may improve the symptoms of HAE-C1-INH.