Open AccessA case of IgG4-related sclerosing disease complicated by sclerosing cholangitis, retroperitoneal fibrosis and orbital pseudotumour
Author(s) -
Kazuki Nagai,
Hiroo Hosaka,
Yutaka Takahashi,
Shunsuke Kubo,
Noriko Nakamura,
Kazuo Andou
Publication year - 2009
Publication title -
bmj case reports
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.231
H-Index - 26
ISSN - 1757-790X
DOI - 10.1136/bcr.02.2009.1590
Subject(s) - medicine , retroperitoneal fibrosis , primary sclerosing cholangitis , pathological , igg4 related disease , disease , fibrosis , rare disease , pathology
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.