WARREN STROBER, M.D.; Z. MYRON FALCHUK, M.D.; G. NICHOLAS ROGENTINE, M.D.; DAVID L. NELSON, M.D.; HAYDEN L. KLAEVEMAN, M.D.
Gluten-sensitive enteropathy is characterized by flattening of intestinal villi and malabsorption caused by the toxic effect of gluten, a wheat protein. Gluten activates an endogenous mechanism of toxicity that may be the local mucosal immune system local mucosal immunoglobulin and antigluten antibody production occur soon after gluten ingestion. Approximately 80% of patients with this disease possess HL-A8, a second segregant series antigen. This association also occurs in dermatitis herpetiformis, a disease with vesicular skin lesions and gluten-sensitive flattening of intestinal villi. The association suggests that the fundamental abnormality in enteropathy is a binding reaction involving gluten protein and a binding site on a cell surface, determined in part by the histocompatibility gene; this reaction then results in a local mucosal immune response to gluten. Alternatively, the fundamental abnormality may be the presence of an abnormal immune-response gene linked to the HL-A8 gene or acting in concert with it; this immune-response gene results in local mucosal production of antigluten antibody.
STROBER W, FALCHUK ZM, ROGENTINE GN, NELSON DL, KLAEVEMAN HL. The Pathogenesis of Gluten-Sensitive Enteropathy. Ann Intern Med. ;83:242–256. doi: 10.7326/0003-4819-83-2-242
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Published: Ann Intern Med. 1975;83(2):242-256.
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