title: [Clinical characteristics of IgG4-related retroperitoneal fibrosis].
abstract: 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.
  
  
  CONCLUSIONS
  IgG4-related RPF is a systemic autoimmune disease with favorable responses to the treatments of glucocorticoid and immunosuppressive agents.
authors.affiliation: Department of Immunology & Rheumatology, Peking Union College Hospital & Chinese Academy of Medical Sciences, Key Laboratory of Immunological & Rheumatologic Diseases under Ministry of Education, Beijing 100032, China.
authors.forename: Hua
authors.initials: H
authors.lastname: Chen
authors.affiliation: Department of Immunology & Rheumatology, Peking Union College Hospital & Chinese Academy of Medical Sciences, Key Laboratory of Immunological & Rheumatologic Diseases under Ministry of Education, Beijing 100032, China.
authors.forename: Wei
authors.initials: W
authors.lastname: Lin
authors.affiliation: Department of Immunology & Rheumatology, Peking Union College Hospital & Chinese Academy of Medical Sciences, Key Laboratory of Immunological & Rheumatologic Diseases under Ministry of Education, Beijing 100032, China.
authors.forename: Linyi
authors.initials: L
authors.lastname: Yan
authors.affiliation: Department of Immunology & Rheumatology, Peking Union College Hospital & Chinese Academy of Medical Sciences, Key Laboratory of Immunological & Rheumatologic Diseases under Ministry of Education, Beijing 100032, China.
authors.forename: Haiting
authors.initials: H
authors.lastname: Zhang
authors.affiliation: Department of Immunology & Rheumatology, Peking Union College Hospital & Chinese Academy of Medical Sciences, Key Laboratory of Immunological & Rheumatologic Diseases under Ministry of Education, Beijing 100032, China.
authors.forename: Qingjun
authors.initials: Q
authors.lastname: Wu
authors.affiliation: Department of Immunology & Rheumatology, Peking Union College Hospital & Chinese Academy of Medical Sciences, Key Laboratory of Immunological & Rheumatologic Diseases under Ministry of Education, Beijing 100032, China.
authors.forename: Li
authors.initials: L
authors.lastname: Wang
authors.affiliation: Department of Immunology & Rheumatology, Peking Union College Hospital & Chinese Academy of Medical Sciences, Key Laboratory of Immunological & Rheumatologic Diseases under Ministry of Education, Beijing 100032, China.
authors.forename: Yunyun
authors.initials: Y
authors.lastname: Fei
authors.affiliation: Department of Immunology & Rheumatology, Peking Union College Hospital & Chinese Academy of Medical Sciences, Key Laboratory of Immunological & Rheumatologic Diseases under Ministry of Education, Beijing 100032, China.
authors.forename: Jinmei
authors.initials: J
authors.lastname: Su
authors.affiliation: Department of Immunology & Rheumatology, Peking Union College Hospital & Chinese Academy of Medical Sciences, Key Laboratory of Immunological & Rheumatologic Diseases under Ministry of Education, Beijing 100032, China.
authors.forename: Qian
authors.initials: Q
authors.lastname: Wang
authors.affiliation: Department of Immunology & Rheumatology, Peking Union College Hospital & Chinese Academy of Medical Sciences, Key Laboratory of Immunological & Rheumatologic Diseases under Ministry of Education, Beijing 100032, China.
authors.forename: Jing
authors.initials: J
authors.lastname: Li
authors.affiliation: Department of Immunology & Rheumatology, Peking Union College Hospital & Chinese Academy of Medical Sciences, Key Laboratory of Immunological & Rheumatologic Diseases under Ministry of Education, Beijing 100032, China. Email: zhangwen91@sina.com.
authors.forename: Wen
authors.initials: W
authors.lastname: Zhang
authors.affiliation: Department of Immunology & Rheumatology, Peking Union College Hospital & Chinese Academy of Medical Sciences, Key Laboratory of Immunological & Rheumatologic Diseases under Ministry of Education, Beijing 100032, China.
authors.forename: Xinping
authors.initials: X
authors.lastname: Tian
authors.affiliation: Department of Immunology & Rheumatology, Peking Union College Hospital & Chinese Academy of Medical Sciences, Key Laboratory of Immunological & Rheumatologic Diseases under Ministry of Education, Beijing 100032, China.
authors.forename: Xuan
authors.initials: X
authors.lastname: Zhang
authors.affiliation: Department of Immunology & Rheumatology, Peking Union College Hospital & Chinese Academy of Medical Sciences, Key Laboratory of Immunological & Rheumatologic Diseases under Ministry of Education, Beijing 100032, China.
authors.forename: Yan
authors.initials: Y
authors.lastname: Zhao
authors.affiliation: Department of Immunology & Rheumatology, Peking Union College Hospital & Chinese Academy of Medical Sciences, Key Laboratory of Immunological & Rheumatologic Diseases under Ministry of Education, Beijing 100032, China.
authors.forename: Xiaofeng
authors.initials: X
authors.lastname: Zeng
authors.affiliation: Department of Immunology & Rheumatology, Peking Union College Hospital & Chinese Academy of Medical Sciences, Key Laboratory of Immunological & Rheumatologic Diseases under Ministry of Education, Beijing 100032, China.
authors.forename: Fengchun
authors.initials: F
authors.lastname: Zhang
mesh_terms: D001327:Autoimmune Diseases; D002681:China; D005260:Female; D005938:Glucocorticoids; D006801:Humans; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D008206:Lymphatic Diseases; D008297:Male; D008875:Middle Aged; D010195:Pancreatitis; D012185:Retroperitoneal Fibrosis; D012793:Sialadenitis
chemical_list: D005938:Glucocorticoids; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents