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BMJ Case Reports 2009; doi:10.1136/bcr.02.2009.1590
  • Rare disease

A case of IgG4-related sclerosing disease complicated by sclerosing cholangitis, retroperitoneal fibrosis and orbital pseudotumour

  1. Kazuki Nagai1,
  2. Hiroo Hosaka2,
  3. Yutaka Takahashi2,
  4. Shuichi Kubo2,
  5. Noriko Nakamura3,
  6. Kazuo Andou4
  1. 1
    Nagai Clinic, Internal Medicine, 1-7-25, Yokodi, Isogo-ku,, Yokohama City, Kanagawa, 2350045, Japan
  2. 2
    Saiseikai Yokohama City Nanbu Hospital, Internal Medicine, 3-2-10, Konandai, Konan-ku, Yokohama City, 233-8503, Japan
  3. 3
    Saiseikai Yokohama City Nanbu Hospital, Pathology, 3-2-10, Konandai, Konan-ku, Yokohama City, Kanagawa, 233-8503, Japan
  4. 4
    Saiseikai Yokohama City Nanbu Hospital, Radiology, 3-2-10, Konandai, Konan-ku, Yokohama City, Kanagawa, 233-8503, Japan
  1. Kazuki Nagai, k-nagai{at}mtj.biglobe.ne.jp
  • Published 1 June 2009

Summary

We present a case of IgG4-related sclerosing disease complicated by sclerosing cholangitis (SC), idiopathic retroperitoneal fibrosis (IRF) and orbital pseudotumour (OPT). Clinical, radiographic and pathological findings later suggested that the patient had SC complicated by IRF. The patient’s SC and IRF were well controlled for the first 10 years of the follow-up period; OPT developed in the tenth year. During investigation of the OPT, serum IgG4 level was found to be significantly elevated. The patient was then diagnosed with IgG4-related sclerosing disease complicated by SC, IRF and OPT. This is a rare manifestation of IgG4-related sclerosing disease, which was diagnosed incidentally during OPT work-up. We suggest that this is a variation of the so-called IgG4-related sclerosing disease or hyper-IgG4 disease.

Footnotes

  • Competing interests: none.

  • Patient consent: Patient/guardian consent was obtained for publication.

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