A rare case report of immunoglobulin G4-related sclerosing mesenteritis and review of the literature

• Published in: Medicine (Baltimore) Vol. 99; no. 41; p. e22579
• Main Authors: Liu, Zhicheng, Jiao, Yan, He, Liang, Wang, Helei, Wang, Daguang
• Format: Journal Article
• Language: English
• Published: United States Lippincott Williams & Wilkins 09.10.2020
• Subjects: Aged; Clinical Case Report; Humans; Immunoglobulin G4-Related Disease - diagnostic imaging; Immunoglobulin G4-Related Disease - surgery; Male; Mesentery - diagnostic imaging; Panniculitis, Peritoneal - diagnostic imaging; Panniculitis, Peritoneal - surgery; Tomography, X-Ray Computed
• ISSN: 0025-7974; 1536-5964
• DOI: 10.1097/MD.0000000000022579

Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a rare autoimmune disorder involving 1 or multiple organs, most commonly the pancreas, lacrimal glands, and salivary glands. However, IgG4-related sclerosing mesenteritis (SM) involving the small-bowel mesentery is rare. Given that IgG4-related SM usually mimics the imaging characteristics of mesenteric malignancies, its preoperative diagnosis remains challenging. In addition, no specific consensus has been reached regarding the treatment of IgG4-related SM. Therefore, a better understanding of the characteristics, treatment, and prognosis of IgG-related SM is urgently needed. Herein, we report a rare case of IgG-related SM. A 67-year-old man was admitted to our hospital after incidental detection of an abdominal mass on ultrasound imaging, although he reported being generally well. The findings on triple-phase abdominal computed tomography were highly consistent with a malignant mesenteric tumor. The hallmark histopathological features along with elevated levels of IgG4 (145 mg/dL) and imaging findings were indicative of IgG-related SM. The patient was treated surgically. Postoperative histopathological examinations exhibited tissue infiltration with lymphocytes and IgG4-positive plasma cells, as well as fibrosis. Ten days after surgery, the patient was discharged from the hospital, and did not show any clinical sign of IgG-related SM within 1-year follow-up. This case highlights the mesentery as an uncommon site of involvement as well as how early IgG-related SM can be completely asymptomatic. Thus, this study has advanced our knowledge of IgG-related SM and may improve treatments for similar conditions.