Purpose: To report natural history and response to treatments of sclerosing mesenteritis. Introduction: Sclerosing mesenteritis is an extremely rare nonneoplastic disease, characterized by fat necrosis, fibrosis, and chronic inflammation. It is in a spectrum of IgG4 sclerosing disease that usually involves pancreas, mesentery, biliary tract, and retroperitoneum. Previous studies revealed inconsistent data of natural history and response to treatment. We report case series diagnosed in our institutions and a systemic review of the disease. Methods: Two reviewers independently conducted a detailed literature search using MeSH terms: sclerosing mesenteritis, mesenteric lipodystrophy, mesenteric panniculitis, and retractile mesenteritis on major electronic databases including PubMed, Ovid Medline, Cochrane Library, and Embase. The reference list of each article was then hand searched. Exclusion criteria are incomplete clinical data, follow up duration of less than 1 month, and lack of histology. We also identified 6 cases from our institutions shown in a Table.Table: [274] Patients with sclerosing mesenteritis at University of Minnesota and VA Minneapolis Medical CenterResults: A total of 248 abstracts were scanned. We identified 25 studies(264 patients, 69% were male, with median age of 63 years) published during 1980 and 2012 to characterize natural history of the disease and 21 studies(138 patients) to evaluate responses to treatments. Presenting symptoms included abdominal pain in 60%, palpable mass in 29%, weight loss in 24%, obstruction in 19%, diarrhea in 16%, and chylous ascites in 6%. Treatment included medical therapy alone in 30%, surgery alone in 31%, surgery followed by medical therapy in 8%, and 31% received no treatment. Among 138 patients, 60% responded to medical therapy alone, 48% responded to surgery therapy alone, and 64% responded to additional medical treatment after surgery. Among patients treated with medical therapy alone, 67% responded to tamoxifen-based regimen(44%) while 48% responded to non-tamoxifen-based regimen(56%). Bowel obstruction was the major indication for surgery. Among patients who received no treatment, 27% resolved spontaneously while 70% had mild persistent symptoms. In our own institutions, 4 cases had mesenteric mass on initial CT abdomen, one had mesenteric mass found on exploratory laparotomy, and the other one presented with chylous pleural effusion. Response to treatment correlated with prior studies. Conclusion: Symptomatic patients may benefit from tamoxifen-based regimen therapy. Additional medical therapy after surgery might improve prognosis.
Read full abstract