Background: 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.
Aim: to provide further insight into RCD and the development of EATL, by reporting on long term survival, risk of transition of RCD into EATL in a large cohort of patients with complicated coeliac disease.
Design and Methods: 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 RCD II who developed EATL after a period of refractoriness to a gluten free diet (secondary EATL) and 13 EATL patients without preceding history of complicated coeliac disease (de novo EATL).
Results: No coeliac disease related mortality is recognized in the RCD I group. The overall five year survival in RCD I is 96%, in RCD II (total) is 58% and in RCD II after developing EATL is only 8%. The 2 year survival in the de novo EATL is 20% versus 15% in secondary EATL (P=0.63).Twenty eight (56%) from 50 patients with RCD-II died, 23 (46%) due to EATL and 4 due to progressive refractory state with emaciation and one from neurocoeliac disease.
Conclusion: Remarkably, no patient with RCD I developed RCD II or EATL within mean follow up of five years (range 2-15 years). Fifty two percent of the RCD II patients developed EATL within 4-6 years after the diagnosis of RCD II. More aggressive and targeted therapies seem necessary in RCD II and EATL.
- Refractory coeliac disease
- stem cell transplantation