Clinical and Immunological Features of Adult-Onset Generalized Autoimmune Gut Disorder

Abstract

We report the second case of an adult fulfilling all diagnostic criteria for GAGD. We propose that the activated LP CD4+ T lymphocytes, as well as those atypically located in the epithelium, may play a pathogenic role. The alphaE/beta7- IEL could constitute a diagnostic marker of intestinal autoimmunity in the cases when autoantibodies are not evidenced, and mucosal TNF-alpha might represent a novel therapeutic target in this severe disease.

Autoimmune Enteropathy Associated with Cessation of Interferon-Alpha Therapy in Chronic Hepatitis C

“…Microscopically, mild to severe villous blunting is present [12,[15][16][17]. Histopathologic evaluation reveals increased mononuclear cells in the lamina propria, increased numbers of crypt apoptotic bodies, and lymphocytic infiltration into deep crypt epithelium with relative paucity of surface lymphocytosis (\40 lymphocytes per 100 epithelial cells) [12,[16][17][18]. Indirect immunofluorescence of the AIE patient's serum on sections of normal human or monkey small intestine commonly shows gut epithelial cell antibodies composed of anti-enterocyte (AE) antibodies and anti-goblet cell (AG) antibodies.…”

Adult autoimmune enteropathy

“…A number of reports in adults [1][2][3][4][5][6][7] , similar to prior publications in children [8][9][10] , have described an entity, termed autoimmune enteropathy (AIE), that appears to involve mainly, but not exclusively, the small intestine [4,7] .…”

The Spectrum of Autoimmune Enteropathy

“…Cryptitis and crypt abscesses have been reported in severe AIE. Crypt enterocytes commonly show an increased expression of HLA-A, -B, -C molecules [8,55,56]. (See Table 2.2; Fig.…”

“…T and B cell function tests, the lymphocytic subsets and polymorphonuclear cell counts are generally normal. Anti-smooth muscle, anti-nuclear and anti-thyroid microsomal autoantibodies have been identified in the course of the disease [5,8,22,60].…”

“…Despite the fact that the mucosal abnormality is primarily confined to the small intestine, the term "generalized autoimmune gut disorder" has been used to describe the association between AIE and autoimmune colitis [8]. Emerging evidence suggests that AIE can be a manifestation of a more diffuse autoimmune disorder of the gastrointestinal system, which comprises gastritis, colitis, hepatitis and pancreatitis with positivity of a variety of autoantibodies, including antiparietal, anti-goblet cell and anti-smooth muscle antibodies [6,[20][21][22].…”