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Sclerosing mesenteritis: A real manifestation or histological mimic of IgG4-related disease?
Mehmet Ozgur Avincsal1,2, Kyoko Otani1, Maki Kanzawa1,3
1Department of Diagnostic Pathology, Kobe University Graduate School of Medicine, Kobe, Japan.
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.
Insights
Sclerosing mesenteritis shares some features with IgG4-related disease (IgG4-RD) but is typically not considered IgG4-RD. Most cases lack key indicators, suggesting it rarely affects this anatomical site.
Area of Science:
- Gastroenterology
- Pathology
- Immunology
Background:
- Sclerosing mesenteritis is a rare condition characterized by inflammatory and fibrotic changes in the mesentery.
- Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory condition with characteristic histological and serological findings.
Observation:
- Three adult male patients with sclerosing mesenteritis presented with large mesenteric masses.
- Histopathology revealed lymphoplasmacytic infiltration, fibrosis, obliterative phlebitis, and storiform fibrosis.
- While IgG4+ plasma cells were increased, the IgG4+/IgG+ ratio and serum IgG4 levels were below typical IgG4-RD criteria.
Findings:
- Sclerosing mesenteritis exhibits some histological overlap with IgG4-RD, including increased IgG4+ plasma cells.
- Crucially, sclerosing mesenteritis cases reviewed did not meet the full diagnostic criteria for IgG4-RD, notably lacking elevated serum IgG4 and multi-organ involvement.
- Two cases demonstrated spontaneous regression, further differentiating them from typical IgG4-RD responses.
Implications:
- Sclerosing mesenteritis is likely a distinct entity from IgG4-RD, despite shared histological features.
- The findings suggest that IgG4-RD rarely involves the mesentery.
- Further research is needed to fully elucidate the pathogenesis and classification of sclerosing mesenteritis.
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