Javed Sheikh1. 1. Harvard Medical School and Division of Allergy and Inflammation, Beth Israel Deaconess Medical Center, Boston, Massachusetts 02215, USA. jsheikh@bidmc.harvard.edu
Abstract
PURPOSE OF REVIEW: Eighty to 90% of patients with chronic urticaria have no specific external cause for their disease, which is therefore labeled 'chronic idiopathic urticaria'. We now know, however, that as many as 30-50% of patients have evidence of an autoantibody to the high-affinity receptor for IgE (FcepsilonRI), which may be pathogenic. The exact prevalence and role of these autoantibodies is still under investigation. RECENT FINDINGS: The frequency of autoantibodies to FcepsilonRI in chronic urticaria has been estimated at 30-50%, but extensive epidemiological studies have not been done. Recent work has confirmed that autoantibodies to FcepsilonRI can be functional, meaning that they can cause histamine release from basophils in vitro. Evidence increasingly suggests that such autoantibodies are also functional in vivo, but conclusive evidence is still lacking. Approximately 50% of cases of urticaria still have no known cause, but recent studies have demonstrated that some of these patients may have intrinsic abnormalities of basophils or mast cells. SUMMARY: The recent evidence that is discussed in this review helps to clarify the role of autoantibodies in some cases of urticaria, but also points towards other non-autoimmune mechanisms that might be pathogenic. Further investigation in these areas will help us to understand the cause of urticaria in cases that are still classified as 'idiopathic'.
PURPOSE OF REVIEW: Eighty to 90% of patients with chronic urticaria have no specific external cause for their disease, which is therefore labeled 'chronic idiopathic urticaria'. We now know, however, that as many as 30-50% of patients have evidence of an autoantibody to the high-affinity receptor for IgE (FcepsilonRI), which may be pathogenic. The exact prevalence and role of these autoantibodies is still under investigation. RECENT FINDINGS: The frequency of autoantibodies to FcepsilonRI in chronic urticaria has been estimated at 30-50%, but extensive epidemiological studies have not been done. Recent work has confirmed that autoantibodies to FcepsilonRI can be functional, meaning that they can cause histamine release from basophils in vitro. Evidence increasingly suggests that such autoantibodies are also functional in vivo, but conclusive evidence is still lacking. Approximately 50% of cases of urticaria still have no known cause, but recent studies have demonstrated that some of these patients may have intrinsic abnormalities of basophils or mast cells. SUMMARY: The recent evidence that is discussed in this review helps to clarify the role of autoantibodies in some cases of urticaria, but also points towards other non-autoimmune mechanisms that might be pathogenic. Further investigation in these areas will help us to understand the cause of urticaria in cases that are still classified as 'idiopathic'.