title: IgG4-related disease in a Chinese cohort: a prospective study.
abstract: OBJECTIVE
  To report the clinical, laboratory, and histological characteristics in a cohort of 28 Chinese patients with immunoglobulin G4-related disease (IgG4-RD).
  
  
  METHODS
  The patients were admitted to, or were out-patients in, Peking Union Medical College Hospital (PUMCH) between January 2011 and May 2012 according to the criteria for IgG4-RD. Clinical presentations, imaging studies, serum Ig subclass assays, and histological examinations were performed in all patients.
  
  
  RESULTS
  The 28 patients (male-to-female ratio 1.8:1) enrolled in this study had a mean age at onset of disease of 51.5 years and the duration of symptoms before diagnosis was 20.4 months; 57% of the patients presented with a history of allergic disease. The most common symptoms were salivary and lacrimal gland swelling, and abdominal pain. Most patients (93%) presented with multiple organ involvement. Elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels were observed in 16 (57%) and seven (25%) patients, respectively. The mean serum IgG4 level was 1142.3 mg/dL (range 149-3870 mg/dL). Eosinophilia was noted in 11 (39%) patients. IgE levels were elevated in all of eight patients examined. Lymphocyte and plasma cell infiltration and IgG4-positive plasma cell infiltration, together with tissue fibrosis, were the predominant histological findings. Most patients (93%) were given prednisone (0.5-0.8 mg/kg/day). Immunosuppressive agents were administered in 19 (68%) patients. Patients were followed for a mean of 6.3 months; efficacy was noted in more than 90% of cases.
  
  
  CONCLUSIONS
  IgG4-RD is a chronic, systemic, multiorgan inflammatory disorder. IgG4-RD patients generally response well to glucocorticoids but the treatment should be individualized.
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Science, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education , Beijing , China.
authors.forename: H
authors.initials: H
authors.lastname: Chen
authors.forename: W
authors.initials: W
authors.lastname: Lin
authors.forename: Q
authors.initials: Q
authors.lastname: Wang
authors.forename: Q
authors.initials: Q
authors.lastname: Wu
authors.forename: L
authors.initials: L
authors.lastname: Wang
authors.forename: Y
authors.initials: Y
authors.lastname: Fei
authors.forename: W
authors.initials: W
authors.lastname: Zheng
authors.forename: G
authors.initials: G
authors.lastname: Fei
authors.forename: P
authors.initials: P
authors.lastname: Li
authors.forename: Y Z
authors.initials: YZ
authors.lastname: Li
authors.forename: W
authors.initials: W
authors.lastname: Zhang
authors.forename: Y
authors.initials: Y
authors.lastname: Zhao
authors.forename: X
authors.initials: X
authors.lastname: Zeng
authors.forename: F
authors.initials: F
authors.lastname: Zhang
mesh_terms: D000328:Adult; D000368:Aged; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D002681:China; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use; D011446:Prospective Studies
chemical_list: D005938:Glucocorticoids; D007074:Immunoglobulin G; D011241:Prednisone