Autoimmunity against type VII collagen in inflammatory bowel disease.
Autoimmunity against type VII collagen, an adhesion molecule of the extracellular matrix in epithelial basement membranes, is causing the rare organ-specific epidermolysis bullosa acquisita (EBA). An intriguing association between EBA and inflammatory bowel disease (IBD) has been extensively documented over the last decades, but, because of the very low incidence of EBA, received little attention from physicians involved in the care of patients with IBD. More recently, autoantibodies against type VII collagen have been detected in up to 68% of IBD patients. Although these findings suggest that chronic intestinal inflammation in IBD predisposes for autoimmunity against type VII collagen, their relevance for the pathogenesis of both IBD and EBA is still unclear. In this review article, the main features of the association between IBD and EBA are presented and pathomechanistic hypotheses as well as future lines of investigation in this area are discussed. Future research should provide new pathomechanistic insights and will likely facilitate the development of more specific and effective immunotherapeutic strategies for both conditions.
Hundorfean
Gheorghe
G
Department of Internal Medicine A, Ernst-Moritz-Arndt University Greifswald, Greifswald, Germany.
Neurath
Markus F
MF
Sitaru
Cassian
C
D000818:Animals; D001323:Autoantibodies / Q000276:immunology; D001324:Autoantigens / Q000276:immunology; D015551:Autoimmunity; D001485:Basement Membrane / Q000378:metabolism; D015815:Cell Adhesion Molecules; D003093:Colitis, Ulcerative / Q000276:immunology / Q000473:pathology; D024143:Collagen Type VII / Q000276:immunology*; D003424:Crohn Disease / Q000276:immunology / Q000473:pathology; D004195:Disease Models, Animal; D016107:Epidermolysis Bullosa Acquisita / Q000276:immunology / Q000473:pathology; D005109:Extracellular Matrix / Q000276:immunology / Q000378:metabolism; D006801:Humans; D007249:Inflammation / Q000473:pathology; D015212:Inflammatory Bowel Diseases / Q000276:immunology* / Q000473:pathology; D051379:Mice; D013601:T-Lymphocytes / Q000276:immunology
D001323:Autoantibodies; D001324:Autoantigens; D015815:Cell Adhesion Molecules; D024143:Collagen Type VII