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 II in comparison with RS type I. Patients with RS type I, however, show similar rates of disease-related complications as well as substantial mortality.

Introduction: Refractory sprue (RS) is a rare malabsorption syndrome, which often requires long-term corticosteroid treatment. Locally acting budesonide could replace systemic corticosteroid therapy and reduce toxicity in patients with RS. Aims: To evaluate the efficacy and toxicity of budesonide in patients with RS. Patients and Methods: Clinical and histological data from patients with RS who received budesonide were analyzed. RS was defined as villous atrophy and malabsorption in spite of a strict gluten-free diet persisting for >6 months or requiring earlier therapeutic intervention. Results: We identified 9 patients (1 with autoimmune enteropathy, 4 with RS type I without and 3 with RS type II with signs of early T cell lymphoma and 1 with CD4-positive sprue-like intestinal T cell lymphoma), who received 9 mg/day of budesonide (range 6–12) for 24 months (1–60), and 7 of whom had an initial treatment with 40 mg/day of prednisolone (30–60) for 4 months (1–144). The initial body mass index was 18 (13.1–22.8) and increased similarly under prednisolone [21.5 (14.9–26.7), p < 0.05] and budesonide therapy [21 (18–27.2), p < 0.05]. The stool frequency per day also decreased similarly from 6 (2–8) to 2 (1–3) and 2 (1–5), each p < 0.05, under prednisolone and budesonide therapy, respectively. Two patients with RS type II did not respond and 7, including all 4 with RS type I, were clinically stable with budesonide therapy. Skin fragility in 1 patient was the only adverse effect of budesonide therapy. Conclusions: Budesonide may be an effective treatment option in patients with RS type I, which can stabilize the clinical condition similar to prednisolone.

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