The development and initial validation of IgG4-related disease damage index: a consensus report from Chinese IgG4-RD Consortium

Peng, Linyi1; Li, Jingna1; Zhou, Jiaxin1; Fei, Yunyun1; Dong, Lingli2; Liu, Yan-Ying3; Zhang, Dingding4; Wang, Yanhong5,6; Chi, Shuhong7; Wang, Fang8; Hou, Yunxia9; Hong, Xiaoping10; Sun, Hongsheng11; Ye, Yujin12; Che, Nan13; Zhang, Rong14; Liu, Changyan15; Ji, Zongfei16; Sun, Wenjia17; Zhao, Cheng18; Ma, Ning19; Lai, Yamin20; Li, Mengtao1; Zhao, Yan1; Zeng, Xiaofeng1; Lu, Liwei21,22; Zhang, Wen1

2024-03-01

10.1136/rmdopen-2023-003938

RMD OPEN   影响因子和分区

2056-5933

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.

Autoimmune Diseases Outcome Assessment, Health Care Severity of Illness Index

SCI

英语

其他

National Key Research and Development Program of China[2022YFC2703104] ; National High Level Hospital Clinical Research Funding["2022-PUMCH-B-013","A-041","C-006"] ; Beijing Natural Science Foundation[7232113] ; National Natural Science Foundation of China["82071839","82271848"] ; Chinese Academy of Medical Sciences Innovation Fund for Medical Sciences["2022-I2M-CT-B-005","2021-1-I2M-003"]

Rheumatology

Rheumatology

WOS:001186502400003

BMJ PUBLISHING GROUP

Web of Science

1.Chinese Acad Med Sci & Peking Union Med Coll, Peking Union Med Coll Hosp,Minist Educ Key Lab, Natl Clin Res Ctr Dermatol & Immunol Dis NCRC DID, Dept Rheumatol & Clin Immunol,State Key Lab Compl, Beijing, Peoples R China
2.Huazhong Univ Sci & Technol, Tongji Hosp, Tongji Med Coll, Dept Rheumatol & Clin Immunol, Wuhan, Hubei, Peoples R China
3.Capital Med Univ, Beijing Friendship Hosp, Dept Rheumatol & Immunol, Beijing, Peoples R China
4.Chinese Acad Med Sci & Peking Union Med Coll, Peking Union Med Coll Hosp, Med Res Ctr, State Key Lab Complex Severe & Rare Dis, Beijing, Peoples R China
5.Chinese Acad Med Sci, Inst Basic Med Sci, Dept Epidemiol & Biostat, Beijing, Peoples R China
6.Peking Union Med Coll, Sch Basic Med, Beijing, Peoples R China
7.Ningxia Med Univ, Gen Hosp, Dept Rheumatol, Yinchuan, Peoples R China
8.Beijing Hosp, Dept Rheumatol, Beijing, Peoples R China
9.Inner Mongolia Med Univ, Affiliated Hosp, Dept Rheumatol, Hohhot, Peoples R China
10.Southern Univ Sci & Technol, Clin Med Coll Jinan Univ 2, Affiliated Hosp 1, Shenzhen Peoples Hosp,Dept Rheumatol & Immunol, Shenzhen, Guangdong, Peoples R China
11.Shandong First Med Univ, Shandong Prov Hosp, Dept Rheumatol & Immunol, Jinan, Peoples R China
12.Sun Yat Sen Univ, Affiliated Hosp 1, Dept Rheumatol, Guangzhou, Peoples R China
13.Nanjing Med Univ, Affiliated Hosp 1, Dept Rheumatol, Nanjing, Peoples R China
14.China Med Univ, Affiliated Hosp 1, Dept Rheumatol & Immunol, Shenyang, Peoples R China
15.Dalian Med Univ, Affiliated Hosp 2, Dept Rheumatol, Dalian, Peoples R China
16.Fudan Univ, Zhongshan Hosp, Dept Rheumatol, Shanghai, Peoples R China
17.Zhejiang Univ, Affiliated Hosp 2, Sch Med, Dept Rheumatol, Hangzhou, Peoples R China
18.Nanjing Univ, Nanjing Drum Tower Hosp, Med Sch, Nanjing, Jiangsu, Peoples R China
19.First Hosp Jilin Univ, Dept Rheumatol, Changchun, Peoples R China
20.Chinese Acad Med Sci & Peking Union Med Coll, Peking Union Med Coll Hosp, Dept Gastroenterol, Beijing, Peoples R China
21.Univ Hong Kong, Dept Pathol, Hong Kong, Peoples R China
22.Univ Hong Kong, Shenzhen Inst Res & Innovat, Hong Kong, Peoples R China

Peng, Linyi,Li, Jingna,Zhou, Jiaxin,et al. The development and initial validation of IgG4-related disease damage index: a consensus report from Chinese IgG4-RD Consortium[J]. RMD OPEN,2024,10(1).

Peng, Linyi.,Li, Jingna.,Zhou, Jiaxin.,Fei, Yunyun.,Dong, Lingli.,...&Zhang, Wen.(2024).The development and initial validation of IgG4-related disease damage index: a consensus report from Chinese IgG4-RD Consortium.RMD OPEN,10(1).

Peng, Linyi,et al."The development and initial validation of IgG4-related disease damage index: a consensus report from Chinese IgG4-RD Consortium".RMD OPEN 10.1(2024).