Azathioprine in refractory sprue: results from a prospective, open-label study

Mauriño, Eduardo; Niveloni, Sonia I.; Pedreira, Silvia C.; Mazure, Roberto M.; Vázquez, Horacio; Reyes, Hugo; Fiorini, Anthony; Smecuol, Edgardo; Cabanne, Ana; Capucchio, M.; Kogan, Zulema; Bai, Julio C.

doi:10.1016/s0002-9270(02)04387-3

Cited by 43 publications

(20 citation statements)

References 34 publications

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“…9, 10, 12, 26 Diarrhea and general status may improve in patients with RCD II after treatment with corticosteroids but histologic healing is rare, and more importantly, progression to the highly-lethal EATL was not prevented in most patients. Other more effective therapeutic options with the aberrant intraepithelial lymphocyte as target and healing of the intestine as well as avoid progression to EATL as primary outcomes are clearly needed for patients with RCD II.…”

Section: Discussionmentioning

confidence: 99%

“…1, 9 Azathioprine is not useful for induction of response because of a delayed onset of action but may be beneficial as steroid-sparing drug in those patients who have side effects or are dependent on high-dose of steroids. 6, 10, 11 Recently, budesonide was found to induce clinical improvement but not necessarily mucosal recovery in most patients with RCD without the side effects associated with systemic active steroids. 12 By contrast, RCD II is usually resistant to any known therapy, and the coexistence of enteropathy-associated T-cell lymphoma (EATL) must be rigorously investigated.…”

Section: Introductionmentioning

confidence: 99%

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Clinical Staging and Survival in Refractory Celiac Disease: A Single Center Experience

et al. 2009

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Background & Aims Refractory celiac disease (RCD) occurs when both symptoms and intestinal damage persist or recur despite strict adherence to a gluten-free diet. In RCD, the immunophenotype of intraepithelial lymphocytes may be normal and polyclonal (RCD I) or abnormal and monoclonal (RCD II). The aim is to describe the clinical characteristics, treatment, and long-term outcome in a large single-center cohort of patients with RCD. Methods We compared the clinical characteristics and outcome in 57 patients with RCD: 42 with RCD I and 15 with RCD II. Results The overall 5-year cumulative survival is 70, 80 and 45 percent in the entire cohort, RCD I, and RCD II respectively (p = 0.07, among subtypes). The drop in survival was more evident during the first two years after the diagnosis. Fifteen (26%) patients died (n=8 with RCD I and n=7 with RCD II). The refractory state itself and enteropathy-associated T-cell lymphoma (EATL) were the most common causes of death, respectively. A new staging system is proposed based on the cumulative effect of five prognostic factors investigated at the time of the refractory state diagnosis: for patients in stages I, II, and III, the 5-year cumulative survival rate was 96, 71, and 19 percent, respectively (p<0.0001). Conclusions RCD is associated with high mortality with RCD II having an especially poor prognosis because of the development of EATL. A new staging model is proposed that may improve the precision of prognosis in patients with RCD.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Clinical Staging and Survival in Refractory Celiac Disease: A Single Center Experience

et al. 2009

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“…Elemental diets, systemic steroids, oral budesonide, oral thioguanines including azathioprine are used in RCDI (and sometimes are beneficial), but have limited benefit in RCDII 277 278. In RCDII, cyclosporine, cladribine and high-dose chemotherapy with autologous stem cell support have been reported; however, therapy must be individualised and include surveillance for EATL 120…”

Section: Gluten-free Dietmentioning

confidence: 99%

Diagnosis and management of adult coeliac disease: guidelines from the British Society of Gastroenterology

Ludvigsson

Bai

Biagi

et al. 2014

Gut

946

1,032

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A multidisciplinary panel of 18 physicians and 3 non-physicians from eight countries (Sweden, UK, Argentina, Australia, Italy, Finland, Norway and the USA) reviewed the literature on diagnosis and management of adult coeliac disease (CD). This paper presents the recommendations of the British Society of Gastroenterology. Areas of controversies were explored through phone meetings and web surveys. Nine working groups examined the following areas of CD diagnosis and management: classification of CD; genetics and immunology; diagnostics; serology and endoscopy; follow-up; gluten-free diet; refractory CD and malignancies; quality of life; novel treatments; patient support; and screening for CD.

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“…Although valuable for treatment of RCD type 1, azathioprine should be used with caution because of potential severe side effects, especially myelosuppression, infections and lymphomagenesis 82 83. Lymphomagenesis is of special concern in patients with RCD type 2 because of the higher risk of EATL development in this subgroup and the fact that monoclonality persists after treatment with azathioprine 68 71 76. Intravenous cladribine (0.1 mg/kg/day for 5 days) was well tolerated in an open-label study in patients with RCD type 2 previously treated with prednisone and/or azathioprine and can induce clinical improvement (36%), histological improvement (59%), and significant decrease in the number of clonal intraepithelial lymphocytes (35%) 84.…”

Section: Alternative Therapiesmentioning

confidence: 99%

Classification and management of refractory coeliac disease

Rubio–Tapia

Murray²

2010

Gut

285

245

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Refractory celiac disease (RCD) is defined by persistent or recurrent malabsorptive symptoms and villous atrophy despite strict adherence to a gluten-free diet (GFD) for at least 6–12 months in the absence of other causes of non-responsive treated celiac disease (CD) and overt malignancy. Symptoms are often severe and require additional therapeutic intervention besides GFD. RCD can be classified as type 1 (normal intraepithelial lymphocyte phenotype), or type 2 (defined by the presence of abnormal [clonal] intraepithelial lymphocyte phenotype). Some patients with RCD may never have responded to a GFD or may have relapsed despite adherence and initial response to the GFD. RCD type 1 usually improves after treatment with a combination of aggressive nutritional support, adherence to GFD, and alternative pharmacologic therapies. By contrast, clinical response to alternative therapies in RCD type 2 is less certain and the prognosis is poor. Severe complications such as ulcerative jejunitis and enteropathy-associated T-cell lymphoma (EATL) may occur in a subgroup of patients with RCD. The aims of this article are (1) to review recent advances in the diagnosis and management of patients with RCD and (2) to describe current and novel methods for classification of patients with RCD into categories that are useful to predict outcome and direct treatment.

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Azathioprine in refractory sprue: results from a prospective, open-label study

Cited by 43 publications

References 34 publications

Clinical Staging and Survival in Refractory Celiac Disease: A Single Center Experience

Clinical Staging and Survival in Refractory Celiac Disease: A Single Center Experience

Diagnosis and management of adult coeliac disease: guidelines from the British Society of Gastroenterology

Classification and management of refractory coeliac disease

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