[Clinical characteristics of IgG4-related retroperitoneal fibrosis].

Zhonghua yi xue za zhi

PubMedID: 25549682

Chen H, Lin W, Yan L, Zhang H, Wu Q, Wang L, Fei Y, Su J, Wang Q, Li J, Zhang W, Tian X, Zhang X, Zhao Y, Zeng X, Zhang F. [Clinical characteristics of IgG4-related retroperitoneal fibrosis]. Zhonghua Yi Xue Za Zhi. 2014;94(39):3079-81.
OBJECTIVE
To explore the clinical characteristics of IgG4-related retroperitoneal fibrosis (RPF) in China.

METHODS
From December 2010 to September 2013, a total of 107 IgG4-related disease (IgG4-RD) patients were reviewed prospectively. Their clinical, laboratory and histopathological features were summarized. And the treatment responses were analyzed.

RESULTS
Among them, 22 RPF cases were identified. The male-to-female ratio and age were 4.5: 1 and 63 ± 11 years respectively. The follow-up period was 3-28 months. The most common onset symptom was low back pain (55%). Multiple organ involvements were documented in 20 patients (91%), including lymphadenopathy (n = 9), aortitis (n = 8), sialadenitis (n = 7), dacryoadenitis (n = 5) and autoimmune pancreatitis (n = 4). Allergic histories were reported by10 subjects (46%). Eosinophilia was present in 6 subjects (27%). Serum IgG4 levels were significantly elevated in all subjects. Serum total IgE level was elevated in 12/13 tested subjects. Tissue biopsies of 12 subjects revealed massive lymphocytic infiltration and obvious tissue fibrosis. Immunohistochemical staining showed a massive infiltration of IgG4-positive plasma cell. Glucocorticoid was administrated in all subjects. Most subjects received combined therapy with immunosuppressive agents or tamoxifen.

CONCLUSION
IgG4-related RPF is a systemic autoimmune disease with favorable responses to the treatments of glucocorticoid and immunosuppressive agents.