title: Clinical characteristics of immunoglobulin G4-related disease: a prospective study of 118 Chinese patients.
abstract: OBJECTIVE
  To characterize the clinical features of IgG4-related disease (IgG4-RD) in China.
  
  
  METHODS
  A prospective cohort study of IgG4-RD was carried out in Peking Union Medical College Hospital between 2011 and 2013. Patients with newly diagnosed IgG4-RD were enrolled.
  
  
  RESULTS
  A total of 118 patients with IgG4-RD were enrolled, including 82 males and 36 females, aged 53.1 (s.d. 13.6) years. The most common symptom at onset was lacrimal gland swelling (38/32.2%). A range of organs were involved: 77 patients (65.3%) had lymphadenopathy, 76 (64.4%) had sialadenitis, 60 (50.8%) had dacryoadenitis, 45 (38.1%) had autoimmune pancreatitis, 32 (27.1%) had pulmonary involvement, 31 (26.3%) had periaortitis/retroperitoneal fibrosis, 29 (35.4% of male patients) had prostatitis and 29 (24.6%) had renal involvement. In addition, there were 21 (17.8%) cases of sclerosing cholangitis, 15 (12.7%) of sinusitis and 10 (8.5%) of inflammatory pseudotumour. Uncommon manifestations included mediastinal fibrosis, skin involvement, sclerosing thyroiditis, hypophysitis, orchitis and colitis. Multiple organ involvement was observed in 93 patients, whereas only 4.2% had only a single organ involved. A history of allergy was reported in 73 (61.9%) patients. The serum IgG4 level was elevated in 97.5% and was correlated with the number of organs involved. Most patients were treated with glucocorticoids alone or in combination with immunosuppressive drugs, and the majority usually improved within 3 months.
  
  
  CONCLUSIONS
  IgG4-RD is a systemic inflammatory and sclerosing disease. Parotid and lacrimal involvement (formerly called Mikulicz's disease), lymphadenopathy and pancreatitis are the most common manifestations. Patients with IgG4-RD showed favourable responses to treatment with glucocorticoids and immunosuppressive agents.
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education.
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authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education.
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authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education.
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authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education.
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authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education.
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authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education.
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authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education.
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authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education.
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authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education.
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authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education.
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authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education.
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authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education.
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authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education.
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authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education.
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mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D044466:Asian People; D002681:China / Q000453:epidemiology; D015331:Cohort Studies; D004359:Drug Therapy, Combination; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015994:Incidence; D008206:Lymphatic Diseases / Q000453:epidemiology / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D008882:Mikulicz' Disease / Q000453:epidemiology / Q000209:etiology*; D010195:Pancreatitis / Q000453:epidemiology / Q000209:etiology*; D011446:Prospective Studies; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications* / Q000188:drug therapy / Q000276:immunology*; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents
keywords: IgG4; IgG4-related disease; Mikulicz’s disease; autoimmune pancreatitis; prospective cohort