Autoantibodies in HAE

PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “C1-inhibitor (C1-INH) autoantibodies in hereditary angioedema. Strong correlation with the severity of disease in C1-INH concentrate naive patients.” (Mol Immunol. 2006 Jun 2). Authors are Varga L, Szeplaki G, Visy B et al, from the 3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary. The presence of autoantibodies to C1-inhibitor (C1-INH-Abs) is a hallmark of acquired C1-inhibitor deficiency. In a prospective study performed between 2001 and 2004 in 95 patients with Type I or II HAE, serum samples were tested for total activity of the classical pathway, C1q, C3, C4 and C1-inhibitor (C1-INH) concentration and activity levels, as well as the presence of IgG, IgA and IgM type anti-C1-inhibitor antibodies (C1-INH-Ab). The levels of the IgM C1-INH-Abs exhibited strong negative correlation to the C1-inhibitor concentration and functional activity, total classical complement pathway activity, and a positive correlation to total IgM concentration. Most probable production of these autoantibodies is the consequence of the activation of complement and other plasma enzyme systems during HAE attacks. To access the full abstract of the article, click here.