Kolkhir P, Muñoz M, Asero R, Ferrer M, Kocatürk E, Metz M, Xiang YK, Maurer M
J Allergy Clin Immunol. 2022 Jun;149(6):1819-1831. doi: 10.1016/j.jaci.2022.04.010. PMID: 35667749.
Chronic spontaneous urticaria (CSU) symptoms include the recurrent spontaneous appearance of wheals and intense itch that may last from hours to days and occur for several years. Some patients develop localized and self-limiting angioedema. These manifestations result from a temporary increase in vascular permeability. Almost 13% of patients with CSU experience angioedema and do not develop wheals.
There are 2 main autoimmune mechanisms for CSU: type I autoimmune (autoallergic) CSU, which is associated with with IgE antibodies against autoantigens; and type IIb autoimmune CSU, which is mediated by autoantibodies that activate mast cells via IgE and FceRI. Type IIb autoimmune CSU is present in almost 10% of patients and is characterized by higher disease severity, concomitant autoimmune diseases, low levels of total IgE, elevated levels of IgG-anti–thyroid peroxidase, basopenia, eosinopenia, poor response to antihistamines and to omalizumab, and a good response to cyclosporine. Some new targeted therapies are under development, such as the anti-IgE, ligelizumab, and the Bruton’s tyrosine kinase inhibitors, fenebrutinib and remibrutinib, and an anti-IL-4Ra, dupilumab.
There are missing some studies on the overlap between autoallergic and type IIb autoimmune CSU and on the optimal management of both types of autoimmune CSU, with easy-to-perform, noninvasive and inexpensive markers to assess the treatment response.
