Sclerosing mesenteritis: A real manifestation or histological mimic of IgG4‐related disease?

Abstract

We present three cases of sclerosing mesenteritis and review the literature to learn whether or not sclerosing mesenteritis is an IgG4-related disease (IgG4-RD). Our patients were all adult males. Their mesenteric masses ranged from 6.5 to 14.5 cm in the greatest diameter. Tissue specimens showed moderate to severe lymphoplasmacytic infiltration with occasional eosinophils against a background of irregular fibrosis. Both obliterative phlebitis and storiform fibrosis were noted in all cases. IgG4+ plasma cells were moderately increased in number (46 to 85 cells/high-power field). However, unlike IgG4-RD, the IgG4+/IgG+ plasma cell ratio was <40% (28% to 35%). Serum IgG4 concentrations were also within the normal range (43.2 to 105 mg/dL; normal range <135 mg/dL). Two biopsy cases showed spontaneous regression on imaging approximately 5 months later. No sclerosing conditions were found in other organs. The literature review identified 11 additional cases of sclerosing mesenteritis with IgG4+ plasma cell infiltration. However, conclusive cases with four characteristic features (high serum IgG4 levels, tissue IgG4 elevation, multi-organ involvement, and effective response to glucocorticoid therapy) have never been reported. In conclusion, although sclerosing mesenteritis shares histological features with IgG4-RD, most cases are less likely to be IgG4-related. IgG4-RD seemingly seldom, if ever, affects this anatomical site.

Surgical management of isolated mesenteric autoimmune disease: addressing the spectrum of IgG4-related disease and sclerosing mesenteritis

“…While some authors have proposed the possibility of concurrent IgG 4 -RD and SM,12 13 no cases of SM in this review were found to fulfil all four of the IgG 4 -RD characteristic features. After examining the histopathological and clinical patterns in this limited cohort of patients, Avinscal et al 5 concluded that while some cases of SM may mimic IgG 4 -RD, IgG 4 -RD rarely affects the mesentery. As conclusions from these small studies and expert opinions are highly variable, the question of whether IgG 4 -RD can include the diagnosis of SM remains unknown.…”

“…Histologically, SM includes degeneration of fat cells leading to areas of calcification, occasional infiltration with lipophages, macrophages, plasma cells and a proliferative of fibroblasts. While IgG 4 -RD can affect any organ,4 many investigators purport that IgG 4 -RD, if ever, affects the small bowel mesentery 5. However, cases of SM in patients with elevated IgG 4 -RD have been reported 10 12 13…”

Autopsy case of sclerosing mesenteritis with ossification and an aggressive clinical course

“…IgG4-related SM was reported by Minato et al 4 Avincsa et al reported three cases of idiopathic SM and suggested that SM shares histological features with IgG4related disease, but most SM cases are less likely to be IgG4-related disease. 5 All four Japanese cases reported thus far had a benign clinical course. 4,5 The tumor spontaneously regressed in two cases and was surgically resected without relapse.…”

“…4,5 The tumor spontaneously regressed in two cases and was surgically resected without relapse. 4,5 The patient in the present case died due to severe diarrhea, renal failure, and intestinal perforation. Superior mesenteric artery syndrome may be caused by SM.…”

Mesenteric Panniculitis in the Elderly - Update on Diagnostic and Therapeutic Approach

“…The segments of the small intestine may show slight thickening and elongation. The most common symptoms include fever, abdominal pain, and malaise [23].…”