title: Inflammatory bowel diseases in patients with adaptive and complement immunodeficiency disorders.
abstract: Crohn's disease and ulcerative colitis are idiopathic chronic inflammatory diseases that primarily affect the gastrointestinal tract. The underlying causes remain poorly understood, but there is a growing body of evidence advocating a likely primary pathogenic role for immunodeficiency in the development of Crohn's lesions. Concordantly, a number of congenital immunodeficiencies disrupting the cellular innate immune system strongly predispose to noninfectious, Crohn's-like inflammatory bowel disease. There are case reports and series suggesting that the same may be true for some of the congenital adaptive and complement immunodeficiencies. This review considers and critiques these potential associations.
authors.affiliation: Department of Medicine, University College London, London, UK. d.marks@ucl.ac.uk
authors.forename: Daniel J B
authors.initials: DJ
authors.lastname: Marks
authors.forename: Craig R
authors.initials: CR
authors.lastname: Seymour
authors.forename: Gavin W
authors.initials: GW
authors.lastname: Sewell
authors.forename: Farooq Z
authors.initials: FZ
authors.lastname: Rahman
authors.forename: Andrew M
authors.initials: AM
authors.lastname: Smith
authors.forename: Sara A
authors.initials: SA
authors.lastname: McCartney
authors.forename: Stuart L
authors.initials: SL
authors.lastname: Bloom
mesh_terms: D056704:Adaptive Immunity; D000818:Animals; D001402:B-Lymphocytes / Q000276:immunology; D003093:Colitis, Ulcerative / Q000235:genetics / Q000276:immunology*; D003165:Complement System Proteins / Q000235:genetics / Q000276:immunology*; D003424:Crohn Disease / Q000235:genetics / Q000276:immunology*; D004198:Disease Susceptibility; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications* / Q000151:congenital*; D051379:Mice; D013601:T-Lymphocytes / Q000276:immunology
chemical_list: D003165:Complement System Proteins