Sclerosing Mesenteritis: Clinical Features, Treatment, and Outcome in Ninety-Two Patients

      Background & Aims: Sclerosing mesenteritis is a rare non-neoplastic disease that affects the small bowel mesentery with chronic fibrosing inflammation. There are few data on the natural history and therapeutic options for this condition. Methods: We performed a retrospective and prospective study to describe the clinical characteristics, therapy, and outcome of all cases of sclerosing mesenteritis diagnosed at the Mayo Clinic, Rochester, from 1982–2005. Results: Ninety-two cases were identified; 70% were male, with a median age of 65 years (interquartile range, 55–72). Common presenting symptoms included abdominal pain in 70%, diarrhea in 25%, and weight loss in 23%. Treatment included medical therapy alone in 26%, surgery alone in 13%, surgery followed by medical therapy in 9%, and 52% received no treatment. Ten percent responded to surgery alone, 20% responded to additional medical treatment after surgery, and 38% responded to medical therapy alone. Tamoxifen in combination with prednisone was used in 20 patients, and 60% improved. Non-tamoxifen–based regimens were used in 12 patients, and 8% improved. Eighteen deaths were noted during the study period, and 17% were attributed to complications of sclerosing mesenteritis or its treatment. Conclusions: Although a relatively benign condition, sclerosing mesenteritis can have a prolonged debilitating course with a fatal outcome. Our results suggest that symptomatic patients might benefit from medical therapy, particularly tamoxifen and prednisone combination treatment. Long-term follow-up is needed to substantiate these results.

      Abbreviations used in this paper:

      CT (computed tomography), IQR (interquartile range), MGUS (monoclonal gammopathy of unknown significance), NHL (non-Hodgkin’s lymphoma), RPF (retroperitoneal fibrosis), SM (sclerosing mesenteritis), SP (sclerosing pancreatitis), TGF-β (transforming growth factor–beta)
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