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IgG4‐related sclerosing mesenteritis: A rare mesenteric disease of unknown etiology
Author(s) -
Minato Hiroshi,
Shimizu Junzo,
Arano Yoshihiko,
Saito Kenichiro,
Masunaga Takaharu,
Sakashita Toshiki,
Nojima Takayuki
Publication year - 2012
Publication title -
pathology international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
eISSN - 1440-1827
pISSN - 1320-5463
DOI - 10.1111/j.1440-1827.2012.02805.x
Subject(s) - igg4 related disease , etiology , medicine , pathology , infiltration (hvac) , fibrosis , retroperitoneal fibrosis , rare disease , lesion , disease , physics , thermodynamics
Sclerosing mesenteritis is a rare inflammatory and fibrosing disorder of unknown etiology, while IgG4‐related disease (IgG4‐RD) consists of mass‐forming, fibroinflammatory lesions characterized by high serum IgG4 levels and tissue infiltration of many IgG4‐positive plasma cells; obliterative phlebitis is common. This report describes a case of sclerosing mesenteritis that was considered a manifestation of IgG4‐RD. A 53‐year‐old man underwent right hemicolectomy because of an ileocecal mass that did not improve with conservative therapy. The ill‐defined fibroinflammatory lesion extended in the mesentery with storiform fibrosis, obliterative phlebitis, and infiltration of many IgG4‐positive plasma cells. The ratio of IgG4‐positive/IgG‐positive cells was 64%, and the ratio of forkhead box protein 3 (FOXP3)‐positive/CD4‐positive cells was elevated (13%). It is likely that at least some cases of sclerosing mesenteritis are a manifestation of IgG4‐RD. It is important to investigate this relationship because steroid therapy may benefit such cases.