The development and initial validation of IgG4-related disease damage index: a consensus report from Chinese IgG4-RD Consortium
Ning Ma,
Wen Zhang,
Xiaofeng Zeng,
Lingli Dong,
Yunyun Fei,
Mengtao Li,
Yan Zhao,
Fang Wang,
Yanhong Wang,
Cheng Zhao,
Yunxia Hou,
Nan Che,
Liwei Lu,
Hongsheng Sun,
Xiaoping Hong,
Zongfei Ji,
Yujin Ye,
Jingna Li,
Linyi Peng,
Jiaxin Zhou,
Yan-Ying Liu,
Shuhong Chi,
Changyan Liu,
Wenjia Sun,
Yamin Lai
Affiliations
Ning Ma
1 Interventional Neuroradiology, Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China
Wen Zhang
Peking Union Medical College Hospital, Beijing, China
Xiaofeng Zeng
Department of Rheumatology and Clinical Immunology, Chinese Academy of Medical Sciences & Peking Union Medical College; National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science & Technology; State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital; Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
Lingli Dong
Tongji Hospital, Tongji Medical Colllege, Huazhong University of Science and Technology, Wuhan, China
Yunyun Fei
Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), State Key Laboratory of Complex Severe and Rare Diseases, The Ministry of Education Key Laboratory, Beijing, China
Mengtao Li
Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China
Yan Zhao
Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), State Key Laboratory of Complex Severe and Rare Diseases, The Ministry of Education Key Laboratory, Beijing, China
Fang Wang
14 Shenzhen People’s Hospital, Shenzhen, Guangdong, China
Yanhong Wang
Peking Union Medical College Hospital, Beijing, China
Cheng Zhao
8The First Affiliated Hospital of Guangxi Medical University, Nanning
Yunxia Hou
Department of Rheumatology, The Affiliated Hospital of Inner Mongolia Medical University, Hohhot, China
Nan Che
Department of Rheumatology, The First Affiliated Hospital of Nanjing Medical University, Nanjing, China
Liwei Lu
Department of Pathology and Shenzhen Institute of Research and Innovation, The University of Hong Kong, Hong Kong, China
Hongsheng Sun
Shandong Provincial Hospital Affiliated to Shandong University, Jinan, China
Xiaoping Hong
Department of Rheumatology and Immunology, The Second Clinical Medical College of Jinan University, The First Affiliated Hospital of Southern University of Science and Technology, Shenzhen People’s Hospital, Shenzhen, Guangdong, China
Zongfei Ji
Department of Rheumatology, Zhongshan Hospital, Fudan University, Shanghai, China
Yujin Ye
Department of Rheumatology, The First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China
Jingna Li
Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), State Key Laboratory of Complex Severe and Rare Diseases, The Ministry of Education Key Laboratory, Beijing, China
Linyi Peng
Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), State Key Laboratory of Complex Severe and Rare Diseases, The Ministry of Education Key Laboratory, Beijing, China
Jiaxin Zhou
Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
Yan-Ying Liu
Department of Rheumatology and Immunology, Beijing Friendship Hospital, Capital Medical University, Beijing, Beijing, China
Shuhong Chi
Department of Rheumatology, General Hospital of Ningxia Medical University, Yinchuan, China
Changyan Liu
Department of Rheumatology, the Second Affiliated Hospital of Dalian Medical University, Dalian, China
Wenjia Sun
Department of Rheumatology, The Second Affiliated Hospital Zhejiang University School of Medicine, Hangzhou, China
Yamin Lai
Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
Objective To develop and conduct an initial validation of the Damage Index for IgG4-related disease (IgG4-RD DI).Methods A draft of index items for assessing organ damages in patients with IgG4-RD was generated by experts from the Chinese IgG4-RD Consortium (CIC). The preliminary DI was refined using the Delphi method, and a final version was generated by consensus. 40 IgG4-RD cases representing four types of clinical scenarios were then selected, each with two time points of assessment for at least 3 years of follow-up. 48 rheumatologists from 35 hospitals nationwide were invited to evaluate organ damage using the CIC IgG4-RD DI. The intraclass correlation coefficient (ICC) and the Kendall-W coefficient of concordance (KW) were used to assess the inter-rater reliability. The criterion validity of IgG4-RD DI was tested by calculating the sensitivity and specificity of raters.Results IgG4-RD DI is a cumulative index consisting of 14 domains of organ systems, including a total of 39 items. The IgG4-RD DI was capable of distinguishing stable and increased damage across the active disease subgroup and stable disease subgroup. In terms of scores at baseline and later observations by all raters, overall consistency in scores at baseline and later observations by all raters was satisfactory. ICC at the two time points was 0.69 and 0.70, and the KW was 0.74 and 0.73, respectively. In subgroup analysis, ICC and KW in all subgroups were over 0.55 and 0.61, respectively. The analysis of criterion validity showed a good performance with a sensitivity of 0.86 (95% CI 0.82 to 0.88), a specificity of 0.79 (95% CI 0.76 to 0.82) and an area under the curve of 0.88 (95% CI 0.85 to 0.91).Conclusion The IgG4-RD DI is a useful approach to analyse disease outcomes, and it has good operability and credibility. It is anticipated that the DI will become a useful tool for therapeutic trials and studies of prognosis in patients with IgG4-RD.
