Autoimmune Enteropathy: A Review and Update of Clinical Management

Gentile, Nicole; Murray, Joseph A.; Pardi, Darrell S.

doi:10.1007/s11894-012-0276-2

Cited by 114 publications

(127 citation statements)

References 31 publications

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“…AE in adults is a rare condition, although increasingly reported 9. The presented cases demonstrate the difficulties in establishing the diagnosis of AE.…”

Section: Discussionmentioning

confidence: 82%

Wireless capsule endoscopy as a tool in diagnosing autoimmune enteropathy

Gram-Kampmann¹,

Lillevang

Detlefsen

et al. 2015

BMJ Case Reports

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Autoimmune enteropathy (AE) is an immune mediated illness of the intestinal mucosa. The cause is unknown, and the diagnosis is based on typical characteristics displayed. There is no gold standard for treatment. We present two adult cases of AE and demonstrate the challenges in establishing the diagnosis. The extensive diagnostic work up excluded other more common causes of protracted diarrhoea. Wireless capsule endoscopy (WCE) displayed universal small intestinal mucosal damage with shortened villi that led to the suspicion of AE in both patients. The diagnosis was confirmed with microscopy, showing shortened villi, villous blunting and hyperplasia of crypts in both patients. In one patient, deep crypt lymphocytosis with minimal intraepithelial lymphocytosis was found as well. Both patients were successfully treated with high-dose immunosuppressant therapy to induce and maintain remission. Use of WCE as a diagnostic tool was invaluable in establishing the diagnosis of AE.

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“…AE in adults is a rare condition, although increasingly reported 9. The presented cases demonstrate the difficulties in establishing the diagnosis of AE.…”

Section: Discussionmentioning

confidence: 82%

Wireless capsule endoscopy as a tool in diagnosing autoimmune enteropathy

Gram-Kampmann¹,

Lillevang

Detlefsen

et al. 2015

BMJ Case Reports

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“…These include almost exclusive small bowel involvement, villous blunting, and atrophy. Differences, though, are that only CD has increased numbers of IELs, and that only CD has a clear association with MHC II [ 54 ]. This, therefore, demonstrates that CD is a unique intertwining between gluten (gliadin)-reactive T cells and alterations to the innate immune system that lead to the development of enteropathy.…”

Section: Non-mhc II Moleculesmentioning

confidence: 98%

Using Animal Models of Celiac Disease to Understand the Role of MHC II

Marietta

Rubio–Tapia

Murray

2013

Clinical Gastroenterology

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Untreated CD patients develop increased numbers of intraepithelial lymphocytes, infl ammatory infi ltration of the lamina propria, intestinal permeability, and in a majority of cases, total or subtotal villous atrophy [ 1 ]. The trigger for the development of CD is gluten, typically derived from wheat, barley, and rye [ 2 ]. Gluten is a group of storage proteins found in wheat, rye, and barley. In wheat, gluten consists of two smaller subgroups of proteins, gliadins, and glutenins. In established CD patients, gliadin-specifi c T cells are more prevalent than glutenin-specifi c T cells [ 3 ]; however, there may be an initial stage of development for all CD patients in which their T cells respond to a greater number of epitopes [ 4 ]. A focusing phenomenon occurs later in which T cells in well-established CD patients are predominantly specifi c for epitopes that are a result of deamidation of gliadin by tissue transglutaminase [ 5 ]. CD is also strongly associated with HLA-DQ2 and HLA-DQ8, wherein 95 % are DQ2+ and most of the remaining are DQ8+ [ 6 ]. This contribution of MHC II is only 40 % of the familial risk, though, leaving 60 % of the genetic risk due to non-HLA genes [ 7 ]. This latter point is of great interest to the researchers that use animal models of CD for a number of reasons.The fi rst reason is that none of the spontaneous animal models seem to have a clear association with specifi c MHC II alleles, as is seen in human CD [ 8 ]. This may be interpreted in a number of ways. One interpretation is that MHC II does not play a vital role in the development of gluten-dependent enteropathy; however, years of clinical research have shown that the HLA molecules are necessary, though not suffi cient, for CD to occur [ 9 ]. Indeed, crucial information has come from T-cell lines derived from the jejunum of CD patients. Studies with these in vitro models using DQ2 and/or DQ8 restricted T cells determined that these intestinally derived T cells

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“…A 21-year-old LRBA deficient female patient with refractory diarrhea is a child from the first cousin related parents. The first presentation of her disease was diarrhea which started immune suppression therapies, however autoimmune entheropathy has been reported with partial response to immunosuppressive drugs such as cyclosporine, azathioprine and 6-mercaptopurine (20). Tacrolimus has been used as a treatment option for enteropathies, especially in autoimmune patients.…”

Section: Case Presentationmentioning

confidence: 99%