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: Hiroshi Minato, Junzo Shimizu, Yoshihiko Arano, Kenichiro Saito, Takaharu Masunaga, Toshiki Sakashita, Takayuki Nojima
• Journal: Pathology international (Pathol Int) Vol. 62 Issue 4 Pg. 281-6 (Apr 2012) ISSN: 1440-1827 [Electronic] Australia
• PMID: 22449233 (Publication Type: Case Reports, Journal Article)
• Copyright: © 2012 The Authors. Pathology International © 2012 Japanese Society of Pathology and Blackwell Publishing Asia Pty Ltd.

Chemical References

• Anti-Bacterial Agents
• Biomarkers
• CD4 Antigens
• FOXP3 protein, human
• Forkhead Transcription Factors
• Immunoglobulin G

Topics

• Anti-Bacterial Agents (therapeutic use)
• Biomarkers (metabolism)
• CD4 Antigens (metabolism)
• Cecum (pathology, surgery)
• Colectomy
• Forkhead Transcription Factors (metabolism)
• Humans
• Ileum (pathology, surgery)
• Immunoglobulin G (metabolism)
• Intestinal Neoplasms (diagnosis, surgery)
• Male
• Mesentery (pathology, surgery)
• Middle Aged
• Panniculitis, Peritoneal (diagnosis, metabolism, therapy)
• Plasma Cells (metabolism, pathology)
• Treatment Outcome