Sclerosing Mesenteritis and IgG4-related Mesenteritis: Case Series and a Systematic Review of Natural History and Response to Treatments

IgG4-related Disease from Head to Toe

“…Although its physiopathology remains un-known, sclerosing mesenteritis is often associated with other fibroinflammatory disorders, including autoimmune pancreatitis, sclerosing cholangitis, and retroperitoneal fibrosis. Recently, Kerdsirichairat et al (73) reported that up to 61% of cases of sclerosing mesenteritis showed histopathologic features of IgG4-related disease such as lymphoplasmacytic infiltration, fibrosis, and obliterative phlebitis. At imaging, sclerosing mesenteritis appears as a soft-tissue mass enveloping the mesenteric vessels, mimicking other mesenteric processes such as lymphoma, carcinoid tumor, and carcinomatosis.…”

IgG4-Related Sclerosing Mesenteritis

“…Histologically, SM shows fibrosis with fat necrosis, chronic inflammation especially around the vessels, and variable focal calcification. In particular, if the immunologic and histologic characteristics are consistent with the IgG4-RD, it is classified as IgG4-RSM [ 3 ]. The histologic features of IgG4-RSM include more than two of the followings: lymphoplasmacytic infiltration, storiform fibrosis, and obliterative phlebitis.…”

“…Kerdsirichairat et al [ 3 ] conducted a systematic literature review of SM with IgG4 testing, and 11 out of 18 cases in total met the criteria for IgG4-RSM. The age of patients with IgG4-RSM ranges from 48 to 82.…”

“…There is no generalized consensus regarding the treatment of SM, including medical therapy, surgical therapy, and surgery with additional medical therapy. In one study, the response rates were shown to be good for all therapies (65%, 82%, and 71%, respectively) [ 3 ], but the limited number of cases made it difficult to determine a treatment of choice. Even, when patients with SM are not treated, they show a high rate of spontaneous resolution [ 5 ].…”

“…Sclerosing mesenteritis (SM) is a rare disease first described by Sulla1 in 1924 under the name “retractile mesenteritis.” SM shows fibrosis with fat necrosis and chronic inflammation on microscopic examination [ 2 ]. In particular, if the histologic and immunologic features are associated with IgG4-related disease (IgG4-RD), the condition is defined as IgG4-related sclerosing mesenteritis (IgG4-RSM) [ 3 ]. Here, we report a case of a 70-yearold woman, who presented with a palpable intra-abdominal mass and vague abdominal pain, and was finally diagnosed with IgG4-RSM based on histology and immunohistochemistry.…”