TY - JOUR
T1 - An International Multispecialty Validation Study of the IgG4-Related Disease Responder Index
AU - Wallace, Zachary S.
AU - Khosroshahi, Arezou
AU - Carruthers, Mollie D.
AU - Perugino, Cory A.
AU - Choi, Hyon
AU - Campochiaro, Corrado
AU - Culver, Emma L.
AU - Cortazar, Frank
AU - Della-torre, Emanuel
AU - Ebbo, Mikael
AU - Fernandes, Ana
AU - Frulloni, Luca
AU - Hart, Phil A.
AU - Karadag, Omer
AU - Kawa, Shigeyuki
AU - Kawano, Mitsuhiro
AU - Kim, Myung Hwan
AU - Lanzillotta, Marco
AU - Matsui, Shoko
AU - Okazaki, Kazuichi
AU - Ryu, Jay H.
AU - Saeki, Takako
AU - Schleinitz, Nicolas
AU - Tanasa, Paula
AU - Umehara, Hisanori
AU - Webster, George
AU - Zhang, Wen
AU - Stone, John H.
N1 - Funding Information:
Xencor had no role in the study design or in the collection, analysis, or interpretation of the data, the writing of the manuscript, or the decision to submit the manuscript for publication. Publication of this article was not contingent upon approval by Xencor.
Funding Information:
Supported by Zencor. Dr. Wallace’s work was supported by a Scientist Development Award from the Rheumatology Research Foundation, an NIH Loan Repayment Award, and grant T32-AR-007258 from the National Institute of Arthritis, Musculoskeletal, and Skin Diseases Training.
Publisher Copyright:
© 2018, American College of Rheumatology
PY - 2018/11
Y1 - 2018/11
N2 - Objective: IgG4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ, leading to organ dysfunction and failure. The IgG4-RD Responder Index (RI) was developed to help investigators assess the efficacy of treatment in a structured manner. The aim of this study was to validate the RI in a multinational investigation. Methods: The RI guides investigators through assessments of disease activity and damage in 25 domains, incorporating higher weights for disease manifestations that require urgent treatment or that worsen despite treatment. After a training exercise, investigators reviewed 12 written IgG4-RD vignettes based on real patients. Investigators calculated both an RI score as well as a physician's global assessment (PhGA) score for each vignette. In a longitudinal assessment, 3 investigators used the RI in 15 patients with newly active disease who were followed up over serial visits after treatment. We assessed interrater and intrarater reliability, precision, validity, and responsiveness. Results: The 26 physician investigators included representatives from 6 specialties and 9 countries. The interrater and intrarater reliability of the RI was strong (0.89 and 0.69, respectively). Correlations (construct validity) between the RI and PhGA were high (Spearman's r = 0.9, P < 0.0001). The RI was sensitive to change (discriminant validity). Following treatment, there was significant improvement in the RI score (mean change 10.5 [95% confidence interval (95% CI) 5.4–12], P < 0.001), which correlated with the change in the PhGA. Urgent disease and damage were captured effectively. Discussion: In this international, multispecialty study, we observed that the RI is a valid and reliable disease activity assessment tool that can be used to measure response to therapy.
AB - Objective: IgG4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ, leading to organ dysfunction and failure. The IgG4-RD Responder Index (RI) was developed to help investigators assess the efficacy of treatment in a structured manner. The aim of this study was to validate the RI in a multinational investigation. Methods: The RI guides investigators through assessments of disease activity and damage in 25 domains, incorporating higher weights for disease manifestations that require urgent treatment or that worsen despite treatment. After a training exercise, investigators reviewed 12 written IgG4-RD vignettes based on real patients. Investigators calculated both an RI score as well as a physician's global assessment (PhGA) score for each vignette. In a longitudinal assessment, 3 investigators used the RI in 15 patients with newly active disease who were followed up over serial visits after treatment. We assessed interrater and intrarater reliability, precision, validity, and responsiveness. Results: The 26 physician investigators included representatives from 6 specialties and 9 countries. The interrater and intrarater reliability of the RI was strong (0.89 and 0.69, respectively). Correlations (construct validity) between the RI and PhGA were high (Spearman's r = 0.9, P < 0.0001). The RI was sensitive to change (discriminant validity). Following treatment, there was significant improvement in the RI score (mean change 10.5 [95% confidence interval (95% CI) 5.4–12], P < 0.001), which correlated with the change in the PhGA. Urgent disease and damage were captured effectively. Discussion: In this international, multispecialty study, we observed that the RI is a valid and reliable disease activity assessment tool that can be used to measure response to therapy.
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U2 - 10.1002/acr.23543
DO - 10.1002/acr.23543
M3 - Article
C2 - 29457382
AN - SCOPUS:85055417294
SN - 2151-464X
VL - 70
SP - 1671
EP - 1678
JO - Arthritis Care and Research
JF - Arthritis Care and Research
IS - 11
ER -