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Retroperitoneal Fibrosis: A Clinicopathologic Study With Respect to Immunoglobulin G4

Zen, Yoh MD* †; Onodera, Manabu MD; Inoue, Dai MD; Kitao, Azusa MD; Matsui, Osamu MD; Nohara, Takahiro MD§; Namiki, Mikio MD§; Kasashima, Satomi MD; Kawashima, Atsuhiro MD; Matsumoto, Yasushi MD; Katayanagi, Kazuyoshi MD; Murata, Tetsuya MD**; Ishizawa, Shin MD††; Hosaka, Noriko MD‡‡; Kuriki, Ken MD§§; Nakanuma, Yasuni MD

The American Journal of Surgical Pathology: December 2009 - Volume 33 - Issue 12 - p 1833-1839
doi: 10.1097/PAS.0b013e3181b72882
Original Articles

The possible involvement of immunoglobulin G4 (IgG4) in the pathogenesis of idiopathic sclerosing lesions has been suggested. In this study, a clinicopathologic analysis was performed to reveal characteristics of retroperitoneal fibrosis relating to IgG4. The study involved 17 patients with retroperitoneal fibrosis. Immunohistochemistry revealed numerous IgG4-positive plasma cell infiltrates in 10 cases (IgG4-related), but only a few positive cells in 7 cases (non-IgG4–related). All patients with IgG4-related retroperitoneal fibrosis were male, whereas all except 1 with unrelated lesions were female. Histologically, eosinophilic infiltration (>5 cells per high-power field) and obliterative phlebitis were commonly observed in IgG4-related lesions. Serologically, serum IgG and IgG4 concentrations were significantly higher in the IgG4-related cases, with the IgG4 concentrations all over 135 mg/dL (the upper limit of the normal range). Steroid therapy was performed in 13 cases, and was effective irrespective of IgG4. Three patients had recurrence during the follow up. Five of 10 IgG4-related cases had sclerosing lesions at other sites. The only tests that reliably distinguish the 2 groups were serum IgG4 levels or IgG4/IgG ratio in the plasma cells in a tissue biopsy. The only major clinical difference was the striking male predominance in IgG4-related cases. In conclusion, this study revealed that retroperitoneal fibrosis could be classified as IgG4-related or not. This distinction seems important to help better characterize the biology/pathogenesis of both groups and better predict the possibility of other IgG4-related processes at other anatomic sites.

*Institute of Liver Studies, King's College Hospital, London, UK

Departments of Pathology


§Urology, Kanazawa University Graduate School of Medicine

Departments of Pathology and Laboratory Medicine

Cardiovascular Surgery, Kanazawa Medical Center

Department of Pathology, Ishikawa Prefectural Central Hospital, Kanazawa

**Department of Pathology and Laboratory Medicine, JA Suzuka General Hospital, Suzuka

††Department of Pathology, School of Medicine, Toyama University, Toyama

‡‡Department of Pathology, Nagano Municipal Hospital, Nagano

§§Department of Pathology, Yaizu City Hospital, Yaizu, Japan

Correspondence: Yoh Zen, MD, Institute of Liver Studies, King's College Hospital, Denmark Hill, London SE5 9RS, UK (e-mail:

© 2009 Lippincott Williams & Wilkins, Inc.