Clinical phenotypes of IgG4-related disease: an analysis of two international cross-sectional cohorts. Issue 3 (5th March 2019)

• Record Type: Journal Article
• Authors: Wallace, Zachary S; Zhang, Yuqing; Perugino, Cory A; Naden, Ray; Choi, Hyon K; Stone, John H
• Other Names: author non-byline.; Akamizu Takashi author non-byline.; Akiyama Mitsuhiro author non-byline.; Bateman Adrian author non-byline.; Blockmans Daniel author non-byline.; Brito-Zeron Pilar author non-byline.; Campochiaro Corrado author non-byline.; Carruthers Mollie author non-byline.; Chari Suresh author non-byline.; Chiba Tsutomu author non-byline.; Choi Hyon author non-byline.; Fernandez Codina Andreu author non-byline.; Cornell Lynn author non-byline.; Culver Emma author non-byline.; Dellatorre Emanuel author non-byline.; Deshpande Vikram author non-byline.; Dicaire Jean-Francois author non-byline.; Dong Lingli author non-byline.; Ebbo Mikael author non-byline.; A Ferry Judith author non-byline.; Fragkoulis George author non-byline.; Frost Fabian author non-byline.; Frulloni Luca author non-byline.; A Hart Phil author non-byline.; Hernandez-Molina Gabriela author non-byline.; Inoue Dai author non-byline.; Keat Karuna author non-byline.; Kamisawa Terumi author non-byline.; Kawa Shigeyuki author non-byline.; Kawano Mitsuhiro author non-byline.; Khosroshahi Arezou author non-byline.; Kobayashi Hiroshi author non-byline.; Kodama Yuzo author non-byline.; Kubo Satoshi author non-byline.; Kubota Kensuke author non-byline.; Lanzillotta Marco author non-byline.; Lerch Markus author non-byline.; Liu Yanying author non-byline.; Löhr Matthias author non-byline.; Marvisi Chiara author non-byline.; Martinez-Valle Ferran author non-byline.; Martin-Nares Eduardo author non-byline.; Masaki Yasufumi author non-byline.; Matsui Shoko author non-byline.; Mizushima Ichiro author non-byline.; P Naden Ray author non-byline.; Nakamura Seiji author non-byline.; Nordeide Jan author non-byline.; Notohara Kenji author non-byline.; Okazaki Kazuichi author non-byline.; Paira Sergio author non-byline.; A Perugino Cory author non-byline.; Popovic Jovan author non-byline.; Ramos-Casals Manel author non-byline.; Rosenbaum James author non-byline.; Ryu Jay author non-byline.; Sato Yasuharu author non-byline.; Sharma Amita author non-byline.; Saeki Takako author non-byline.; Sekiguchi Hiroshi author non-byline.; Schleinitz Nicolas author non-byline.; V Sokol Evgeniya author non-byline.; H Stone John author non-byline.; R Stone James author non-byline.; Takahashi Hiroki author non-byline.; Takahashi Naoki author non-byline.; Takahira Masayuki author non-byline.; Tanaka Yoshiya author non-byline.; Umehara Hisanori author non-byline.; Vaglio Augusto author non-byline.; Villamil Alejandra author non-byline.; Wada Yoko author non-byline.; S Wallace Zachary author non-byline.; Webster George author non-byline.; Yamada Kazunori author non-byline.; Yamamoto Motohisa author non-byline.; Yi Joanne author non-byline.; Zamboni Giuseppe author non-byline.; Zen Yoh author non-byline.; Zhang Wen author non-byline.;
• Is Part Of: Annals of the rheumatic diseases. Volume 78:Issue 3(2019)
• Page Start: 406
• Publication Date: 2019-03-05
• Subjects: IgG4-related disease -- epidemiology -- cluster analysis; Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2018-214603
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : Objective: IgG4-related disease (IgG4-RD) is a heterogeneous, multiorgan condition of unclear aetiology that can cause organ failure. Difficulty recognising IgG4-RD contributes to diagnostic delays. We sought to identify key IgG4-RD phenotypes. Methods: We used two cross-sectional studies assembled by an international, multispecialty network of IgG4-RD specialists who submitted 765 cases to derive and replicate phenotypic groups. Phenotype groups of disease manifestations and key covariate distributions across the identified groups were measured using latent class analysis. Results: In the derivation cohort (n=493), we identified four groups with distinct manifestations: Group 1 (31%), Pancreato-Hepato-Biliary disease; Group 2 (24%), Retroperitoneal Fibrosis and/or Aortitis; Group 3 (24%), Head and Neck-Limited disease and Group 4 (22%), classic Mikulicz syndrome with systemic involvement. We replicated the identification of four phenotype groups in the replication cohort. Compared with cases in Groups 1, 2 and 4, respectively, cases in Group 3 were more likely to be female (OR 11.60 (95% CI 5.39 to 24.98), 10.35 (95% CI 4.63 to 23.15) and 9.24 (95% CI 3.53 to 24.20)) and Asian (OR 6.68 (95% CI 2.82 to 15.79), 7.43 (95% CI 2.97 to 18.56) and 6.27 (95% CI 2.27 to 17.29)). Cases in Group 4 had a higher median serum IgG4 concentration (1170 mg/dL) compared with groups 1–3 (316, 178 and 445 mg/dL, respectively, p<0.001). Conclusion: We identified four distinctive IgG4-RD phenotypes according to organ involvement. Being Asian or female may predispose individuals to head and neck-limited disease. These phenotypes serve as a framework for identifying IgG4-RD and studying its aetiology and optimal treatment.