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

Presented at Digestive Diseases Week, 2003, Orlando, Florida, Digestive Diseases Week, 2006, Los Angeles, CA, and published in abstract form (Gastroenterology 2003;124(Suppl 1):S1297 and Gastroenterology 2006;130(Suppl 2):M1196).
https://doi.org/10.1016/j.cgh.2007.02.032Get rights and content

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.

Section snippets

Methods

After approval by the Mayo Clinic’s Institutional Review Board, 65 patients with a diagnosis of SM and its variants were retrospectively identified from January 1982–November 2002 through the Mayo Clinic diagnostic index and Department of Pathology database. An additional 28 cases were prospectively identified as referrals to our gastroenterology outpatient clinic between December 2002–November 2005. One patient denied research authorization and was excluded from the study. All pathology slides

Clinical Presentation and Diagnosis

Ninety-two cases of SM were identified during the study period. Patient characteristics at presentation are shown in Table 1. The most frequent presenting symptoms were abdominal pain in 70%, bloating and distention in 26%, diarrhea in 25%, and weight loss in 23% (Table 2). SM was an incidental finding in 10% of cases when an abdominal surgery (3%), computed tomography (CT) scan (5%), or autopsy (1%) was performed for another indication, and there were no symptoms attributable to mesenteric

Discussion

The current study represents the largest series of patients reported to date with the diagnosis of SM or its variants and, to the best of our knowledge, the most comprehensive information on treatment responses currently available. Other than 2 prior large series including 53 and 84 patients, respectively,1, 3 most published reports are single patients or small case series8, 12, 13, 14 or reviews of existing literature.4, 5, 6, 15 Because of the paucity of published cases, the clinical and

Conclusions

SM is a rare idiopathic disorder that involves the small bowel mesentery with varying degrees of fibrosis, inflammation, and fat necrosis. Its clinical presentation is quite variable and ranges from asymptomatic to debilitating disease. Approximately 50% of the patients might not require any treatment. However, in symptomatic cases, treatment should be tailored according to the severity and type of individual symptoms. Patients with intractable bowel obstruction might require surgery, and those

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