High rates of complications and substantial mortality in both types of refractory sprue

Introduction Refractory sprue (RS) is a rare malabsorption syndrome defined by persisting small bowel villous atrophy despite a strict gluten-free diet The clinical picture and long-term outcome of RS is highly variable and is not well described. Aim To define underlying and accompanying diseases and clinical outcome in consecutive patients with RS. Patients and methods Clinical and histological data from patients with RS at our department were analyzed retrospectively. RS was defined as villous atrophy and malabsorption despite a strict gluten-free diet persisting without improvement for more than 6 months or requiring earlier therapeutic intervention. Results Thirty-two patients with RS were identified (23 RS type I, nine RS type II). Follow-up period was 55 (12-372) months. Two patients progressed from RS type I into type II. Thrombembolic events occurred in nine cases, and additional autoimmune diseases were found in 17 patients. Overt intestinal T-cell lymphoma developed in four patients with RS type II. Three patients with RS type II died during the observation period owing to intestinal T-cell lymphoma and four with RS type I owing to infectious complications. Five-year cumulative survival was 90% (95% confidence interval 76-100) in patients with RS type I and higher than in patients with RS type II (53%, 12-94%; P<0.05). Conclusion RS comprises a very heterogenous group of patients with long-term survival seen even in single patients with RS type II. Overall, survival is shorter in RS type I I in comparison with RS type I. Patients with RS type I, however, show similar rates of disease-related complications as well as substantial mortality. Eur J Gastroenteroi Hepatol 21:66-70 (C) 2009 Wolters Kluwer Health I Lippincott Williams & Wilkins.