title: IgG4-related sclerosing mesenteritis: a rare mesenteric disease of unknown etiology.
abstract: 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.
authors.affiliation: Department of Pathology and Laboratory Medicine, Kanazawa Medical University, Ishikawa, Japan. hminato@kanazawa-med.ac.jp
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Minato
authors.forename: Junzo
authors.initials: J
authors.lastname: Shimizu
authors.forename: Yoshihiko
authors.initials: Y
authors.lastname: Arano
authors.forename: Kenichiro
authors.initials: K
authors.lastname: Saito
authors.forename: Takaharu
authors.initials: T
authors.lastname: Masunaga
authors.forename: Toshiki
authors.initials: T
authors.lastname: Sakashita
authors.forename: Takayuki
authors.initials: T
authors.lastname: Nojima
mesh_terms: D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D015415:Biomarkers / Q000378:metabolism; D015704:CD4 Antigens / Q000378:metabolism; D002432:Cecum / Q000473:pathology / Q000601:surgery; D003082:Colectomy; D051858:Forkhead Transcription Factors / Q000378:metabolism; D006801:Humans; D007082:Ileum / Q000473:pathology / Q000601:surgery; D007074:Immunoglobulin G / Q000378:metabolism*; D007414:Intestinal Neoplasms / Q000175:diagnosis / Q000601:surgery; D008297:Male; D008643:Mesentery / Q000473:pathology / Q000601:surgery; D008875:Middle Aged; D015436:Panniculitis, Peritoneal / Q000175:diagnosis* / Q000378:metabolism* / Q000628:therapy; D010950:Plasma Cells / Q000378:metabolism / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D000900:Anti-Bacterial Agents; D015415:Biomarkers; D015704:CD4 Antigens; C418974:FOXP3 protein, human; D051858:Forkhead Transcription Factors; D007074:Immunoglobulin G