{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,21]],"date-time":"2026-10-21T16:58:47Z","timestamp":1792601927669,"version":"4.5.0"},"reference-count":46,"publisher":"BMJ","issue":"10","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Gut"],"published-print":{"date-parts":[[2007,10]]},"abstract":"<jats:p>\n                  <jats:bold>Background:<\/jats:bold> Coeliac disease may be regarded as refractory disease (RCD) when symptoms persist or recur despite strict adherence to a gluten-free diet. RCD may be subdivided into types I and II with a phenotypically normal and aberrant intraepithelial T-cell population, respectively. RCD I seems to respond well to azathioprine\/prednisone therapy. RCD II is usually resistant to any known therapy and transition into enteropathy-associated T-cell lymphoma (EATL) is common.<\/jats:p>\n               <jats:p>\n                  <jats:bold>Aim:<\/jats:bold> To provide further insight into RCD and the development of EATL, by reporting on long-term survival and risk of transition of RCD into EATL in a large cohort of patients with complicated coeliac disease.<\/jats:p>\n               <jats:p>\n                  <jats:bold>Design and Methods:<\/jats:bold> Retrospective comparison of responses to therapy in four groups of patients with complicated coeliac disease: 43, RCD I; 50, RCD II (total), of whom 26 with RCD II developed EATL after a period of refractoriness to a gluten-free diet (secondary EATL) and 13 were EATL patients without preceding history of complicated coeliac disease (de novo EATL).<\/jats:p>\n               <jats:p>\n                  <jats:bold>Results:<\/jats:bold> No coeliac-disease-related mortality was recognised in the RCD I group. The overall 5-year survival in the RCD I group it was 96%; in the RCD II (total) group was 58%; and in the RCD II group after developing EATL it was only 8%. The 2-year survival in the de novo EATL group was 20% versus 15% in secondary EATL group (p\u200a=\u200a0.63). Twenty-eight (56%) of the 50 patients with RCD II died, 23 (46%) due to EATL, 4 due to a progressive refractory state with emaciation and 1 from neurocoeliac disease.<\/jats:p>\n               <jats:p>\n                  <jats:bold>Conclusion:<\/jats:bold> Remarkably, no patient with RCD I developed RCD II or EATL within the mean follow-up period of 5 years (range 2\u201315 years). A total of 52% of the RCD II patients developed EATL within 4\u20136 years after the diagnosis of RCD II. More aggressive and targeted therapies seem necessary in RCD II and EATL.<\/jats:p>","DOI":"10.1136\/gut.2006.114512","type":"journal-article","created":{"date-parts":[[2007,4,30]],"date-time":"2007-04-30T20:35:34Z","timestamp":1177965334000},"page":"1373-1378","source":"Crossref","is-referenced-by-count":290,"title":["Survival in refractory coeliac disease and enteropathy-associated T-cell lymphoma: retrospective evaluation of single-centre experience"],"prefix":"10.1136","volume":"56","author":[{"given":"A","family":"Al-toma","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"W H M","family":"Verbeek","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M","family":"Hadithi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B M E","family":"von Blomberg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C J J","family":"Mulder","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2007,4,30]]},"reference":[{"key":"2025090715084162000_56.10.1373.1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bpg.2005.02.001"},{"key":"2025090715084162000_56.10.1373.2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(00)02481-8"},{"key":"2025090715084162000_56.10.1373.3","doi-asserted-by":"crossref","unstructured":"Wahab P J, Meijer J W, Goerres M S, et al. 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