ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 2101

The Clinical Characteristics of IgG4 Related Disease in China: With 346 Cases Reported

Panpan Zhang1, Wen Zhang2, Jizhi Zhao3, Mu Wang3, Ruie Feng3, Xiaowei Liu3, Xuemei Li3, Yamin Lai3, Xuejun Zeng3, Juhong Shi3, Huijuan Zhu3, Huadan Xue3, Wei Zhang3, Hua Chen4, Yunyun Fei3, Linyi Peng3, Xiaofeng Zeng5 and Fengchun Zhang3, 1Department of Rheumatology, Peking Union Medical College Hospital, Beijing, China, 2Rheuamtology, Peking Union Medical College Hospital, Beijing, China, 3Peking Union Medical College Hospital, Beijing, China, 4Rheumatology, Peking Union Medical College Hospital, Beijing, China, 5Rheumatology, Peking Union Medical College and Chinese Academy of Medical Sciences, Peking Union Medical College Hospital, Beijing, China

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords:

Session Information

Date: Tuesday, November 7, 2017

Title: Miscellaneous Rheumatic and Inflammatory Diseases Poster II

Session Type: ACR Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose: To study the clinical characteristics of IgG4-RD patients in China

Methods: Patients were recruited from a prospective cohort study of IgG4-RD in Peking Union Medical College Hospital between 2011 and 2016, who were followed-up for more than 6 months. The demographic characteristics, organ involvements, the laboratory examinations and treatment were analyzed

Results: A total number of 346 patients were enrolled, including male 230 (66.5%), female 116 (33.5%).The age of disease onset was 53.8 ± 14.2 years old. The mostly common related organ onset was lymphadenopathy (56.4%), submandibular glands (52.6%). Other affected organs included: swelling of the lacrimal glands (46.5%), autoimmune pancreatitis (38.4%), pulmonary involvement (28.0%), sclerosing cholangitis (25.4%), naso-sinusitis (23.4%), salivary swelling were (21.7%), retroperitoneal fibrosis (19.9%), large arteries involvement (9.5%), kidney involvement (6.9%), skin lesions (6.4%), Rarely involved organs were thyroid glands, pituitary glands, gastrointestinal tract, pachymeningitis, pericardium, sclerosing mediastinitis and orchid. The majority of patients had multi-organ involvement, 74.3% of patients with 3 and more organs involvement 18.2% and 7.5% of patients with 2 and single organ involvement respectively. The average IgG4-RD responder index (IgG4-RD RI) was 13.21±5.7. History of allergy was found in 172(49.7%) patients. Of the laboratory tests, elevated serum IgG4 levels were found in 285(94.1%) patients, and positively correlated with IgG4-RD RI. Of treatment, 33.5% patients received treatment of glucocorticoids, 52.6% patients were treated with glucocorticoids combined with immunosuppressors, 3.2% patients were treated with immunosuppressors, and 9.0% patients received no drugs at baseline evaluation. Most patients improved after regular drug treatment.

Conclusion: IgG4-RD is a systemic fibro-inflammatory disease with multiple organs involvement. The mostly common related organs respectively were lymph node, submandibular glands, and pancreas. Glucocorticoids and immunosuppressors were effective treatment strategies of IgG4-RD.

Disclosure: P. Zhang, None; W. Zhang, None; J. Zhao, None; M. Wang, None; R. Feng, None; X. Liu, None; X. Li, None; Y. Lai, None; X. Zeng, None; J. Shi, None; H. Zhu, None; H. Xue, None; W. Zhang, None; H. Chen, None; Y. Fei, None; L. Peng, None; X. Zeng, None; F. Zhang, Xian Janssen, 8.

To cite this abstract in AMA style:

Zhang P, Zhang W, Zhao J, Wang M, Feng R, Liu X, Li X, Lai Y, Zeng X, Shi J, Zhu H, Xue H, Zhang W, Chen H, Fei Y, Peng L, Zeng X, Zhang F. The Clinical Characteristics of IgG4 Related Disease in China: With 346 Cases Reported [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/the-clinical-characteristics-of-igg4-related-disease-in-china-with-346-cases-reported/. Accessed .

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