Sclerosing mesenteritis is a rare inflammatory disease of the bowel mesentery. It produces tumor-like masses of the mesentery composed of varying degrees of fibrosis, chronic inflammation, and fat necrosis. It has been described variously as fibrosing mesenteritis, retractile mesenteritis, mesenteric Weber Christian disease, and systemic nodular panniculitis. The etiology and pathogenesis of the disease are as yet unknown, but autoimmune disorder, previous abdominal surgery, trauma, and ischemia could play a role. The clinical features include abdominal pain, vomiting, diarrhea, and constipation. Occasionally, patients with this condition may present with bowel obstruction. Rarely, It can be associated with other idiopathic inflammatory disorders such as retroperitoneal fibrosis, sclerosing cholangitis, and orbital pseudotumors. We report a case of idiopathic sclerosing mesenteritis with retroperitoneal fibrosis in a 58-year-old man.
Anti-Inflammatory Agents/therapeutic use ; Antineoplastic Agents, Hormonal/therapeutic use ; Diagnosis, Differential ; Humans ; Laparoscopy ; Male ; Middle Aged ; Panniculitis, Peritoneal/complications/*diagnosis/drug therapy ; Prednisolone/therapeutic use ; Retroperitoneal Fibrosis/complications/*diagnosis/pathology ; Tamoxifen/therapeutic use ; Tomography, X-Ray Computed

Sclerosing mesenteritis (SM) is a rare disease characterized by chronic nonspecific mesenteric inflammation and fibrosis of unknown etiology. Some tumefactive SM shows diffuse accumulation of IgG4-positive plasma cells and is considered as a part of the spectrum of IgG4-related disease. An association between inflammatory bowel disease and IgG4-related disease has been indicated. A 45-year-old woman visited our hospital due to weight loss with intermittent lower abdominal discomfort. Pelvic ultrasound revealed a mass-like lesion in the abdominal wall and pelvis MRI demonstrated a 5.9 cm sized wall-enhancing mass with heterogeneous signal intensity from right adnexa to the abdominal wall. Tumor resection and adhesiolysis was done because of severe adhesion with the small bowel, colon, bladder, uterus, and abdominal wall. Appendectomy was also performed due to adhesion and edematous change. Histological examination of the resected mass showed findings that were compatible with IgG4-related SM. The resected appendix showed chronic granulomatous inflammation without evidence of tuberculosis. She was diagnosed with Crohn's disease after undergoing colonoscopy and CT enterography. Herein, we report a rare case of IgG4-related SM that occurred in conjunction with Crohn's disease.
Anti-Inflammatory Agents/therapeutic use ; Appendix/pathology ; Azathioprine/therapeutic use ; Colonoscopy ; Crohn Disease/complications/*diagnosis/drug therapy ; Female ; Humans ; Immunoglobulin G/*blood ; Magnetic Resonance Imaging ; Mesalamine/therapeutic use ; Middle Aged ; Panniculitis, Peritoneal/*diagnosis/etiology/ultrasonography ; Prednisolone/therapeutic use ; Tomography, X-Ray Computed ; Urinary Bladder/pathology