Clinical characteristics of immunoglobulin G4–related disease: a prospective study of 118 Chinese patients

• Published in: Rheumatology (Oxford, England) Vol. 54; no. 11; pp. 1982 - 1990
• Main Authors: Lin, Wei, Lu, Sha, Chen, Hua, Wu, Qingjun, Fei, Yunyun, Li, Mengtao, Zhang, Xuan, Tian, Xinping, Zheng, Wenjie, Leng, Xiaomei, Xu, Dong, Wang, Qian, Shen, Min, Wang, Li, Li, Jing, Wu, Di, Zhao, Lidan, Wu, Chanyuan, Yang, Yunjiao, Peng, Linyi, Zhou, Jiaxin, Wang, Yu, Sha, Yue, Huang, Xiaoming, Jiao, Yang, Zeng, Xuejun, Shi, Qun, Li, Ping, Zhang, Shulan, Hu, Chaojun, Deng, Chuiwen, Li, Yongzhe, Zhang, Shangzhu, Liu, Jinjing, Su, Jinmei, Hou, Yong, Jiang, Ying, You, Xin, Zhang, Haiting, Yan, Linyi, Zhang, Wen, Zhao, Yan, Zeng, Xiaofeng, Zhang, Fengchun, Lipsky, Peter E.
• Format: Journal Article
• Language: English
• Published: England 01.11.2015
• Subjects: Adult; Aged; Aged, 80 and over; Asian Continental Ancestry Group; China - epidemiology; Cohort Studies; Drug Therapy, Combination; Female; Glucocorticoids - therapeutic use; Humans; Immunoglobulin G; Immunosuppressive Agents - therapeutic use; Incidence; Lymphatic Diseases - epidemiology; Lymphatic Diseases - etiology; Male; Middle Aged; Mikulicz' Disease - epidemiology; Mikulicz' Disease - etiology; Pancreatitis - epidemiology; Pancreatitis - etiology; Prospective Studies; Retrospective Studies; Scleroderma, Systemic - complications; Scleroderma, Systemic - drug therapy; Scleroderma, Systemic - immunology; Treatment Outcome; China; IgG4; autoimmune pancreatitis; prospective cohort; IgG4-related disease; Mikulicz’s disease
• ISSN: 1462-0324; 1462-0332
• DOI: 10.1093/rheumatology/kev203

To characterize the clinical features of IgG4-related disease (IgG4-RD) in China. 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. 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. 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.