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.