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home - Small Bowel - Coeliac Disease - Refractory Coeliac Disease Written by Dr Sebastian Zeki

Refractory Coeliac Disease

Written by Dr Sebastian Zeki Therapeutic Options for Refractory CD:Strict gluten-free diet.Elemental Diet- only anecdotal evidence for this.Total Parenteral Nutrition- can be useful.Steroids-This can be successful in patients who do not have the abnormal clonal IEL phenotype (described previously).Steroids usually 1 month of treatment then taper.Interferon-alpha- not useful.Azathioprine.-a variable outcome.Cyclosporine- one study shows it may be useful. Imaging Studies:CT. EndoscopyMay miss ulcerative jejunoileitis on routine OGD. Preferable to do OGD and colonoscopy at same sitting. Molecular StudiesIn patients with uncomplicated CD, IELs express CD3+, CD8+ (this is predominant), gamma/delta, and alpha/beta T-cell receptors (TCR).In RS there are abnormal CD3 chain(c) + CD8- IEL phenotype and TCR-gamma gene rearrange-ments.75% of patients with RS have an immunopheno-typically aberrant clonal intraepithelial T-cell population. Histology Colon. There is a strong association between RS and lymphocytic colitis, the T-cells of which dont show the normal CD8+ marker usually seen in this condition. Duodenum. Subcryptal chronic inflammation Marked mucosal thinning Subepithelial collagen deposition Jejunum.Ulcerative jejunoileitis only present in 30%- diagnosis with enteroscopy only Diagnosis And Management of Refractory Coeliac DiseaseBoth RS and EATL are characterized by the presence of a monoclonal T-cell population in the intestinal mucosa. In EATL, a similar monoclonal population that shows clonal identity with the lymphoma itself is also present in the enteropathic mucosa. Other patients have evident mucosal atrophy on endoscopy.

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