{"type":"rich","version":"1.0","provider_name":"Transistor","provider_url":"https://transistor.fm","author_name":"Board Pearls","title":"Chapter 10, Ep 2 of 2: Refractory Celiac and Complications","html":"<iframe width=\"100%\" height=\"180\" frameborder=\"no\" scrolling=\"no\" seamless src=\"https://share.transistor.fm/e/8bc6156f\"></iframe>","width":"100%","height":180,"duration":707,"description":"Most celiac patients failing a gluten-free diet are not refractory; they are eating hidden gluten or have a coexisting condition, so a structured non-responsive differential precedes any refractory workup. When refractory disease is genuine, the type one versus type two split on intraepithelial lymphocyte phenotype separates a steroid-responsive disease with good survival from a pre-lymphoma syndrome. The malignancy spectrum, enteropathy-associated T-cell lymphoma, ulcerative jejunitis, and small-bowel adenocarcinoma, is the payoff of getting that distinction right.\n \nTopics covered\n\nNon-responsive celiac differential\nRefractory celiac type 1 vs type 2\nIntraepithelial lymphocyte phenotyping\nEnteropathy-associated T-cell lymphoma\nUlcerative jejunitis\nSmall-bowel adenocarcinoma\nSmall-bowel imaging and surveillance\n \n \nKey decisions\n\nPersistent symptoms on a gluten-free diet are most often ongoing gluten exposure; the first step is a dietitian reviewing the diet line by line, not a refractory workup or steroids.\nWork the non-responsive differential in order: incomplete gluten avoidance, incorrect original diagnosis, coexisting condition (microscopic colitis, pancreatic insufficiency, overgrowth, IBS), then drug-induced enteropathy (olmesartan, NSAIDs, mycophenolate) before invoking refractory disease.\nSend duodenal biopsies for flow cytometry in saline or tissue medium, not formalin, because formalin destroys the surface markers; take extra samples for histology and T-cell clonality.\nType 1: polyclonal, surface CD3 and CD8 retained, abnormal fraction under 20 percent, treated with open-capsule budesonide, good prognosis; check TPMT before a thiopurine.\nType 2: over 20 percent abnormal clonal lymphocytes with loss of surface CD3 and CD8 but retained cytoplasmic CD3, monoclonal clonality, a pre-lymphoma diagnosis treated with cladribine and autologous transplant at a tertiary center.\nWorsening of a stable celiac course on a verified diet, even with negative...","thumbnail_url":"https://img.transistorcdn.com/-FuAdDBcPDLhEoUmroZKtOBRvuBn_FHPpYlh41hOnU4/rs:fill:0:0:1/w:400/h:400/q:60/mb:500000/aHR0cHM6Ly9pbWct/dXBsb2FkLXByb2R1/Y3Rpb24udHJhbnNp/c3Rvci5mbS9iNzlh/ZTU4Y2MzNWExMjQ5/MjA5OWMwMmI3ZTk5/NGFiZS5wbmc.webp","thumbnail_width":300,"thumbnail_height":300}