Clinical phenotypes of IgG4-related disease: an analysis of two international cross-sectional cohorts

Autor: Wallace, Zs, Zhang, Y, Perugino, Ca, Naden, R, Choi, Hk, Stone, Jh, ACR/EULAR IgG4-RD Classification Criteria Committee, Takashi, Akamizu, Mitsuhiro, Akiyama, Adrian, Bateman, Daniel, Blockmans, Pilar, Brito-Zeron, Corrado, Campochiaro, Mollie, Carruthers, Suresh, Chari, Tsutomu, Chiba, Hyon, Choi, Andreu Fernandez Codina, Lynn, Cornell, Emma, Culver, Emanuel, Della-Torre, Vikram, Deshpande, Jean-Francois, Dicaire, Lingli, Dong, Mikael, Ebbo, Judith, A Ferry, George, Fragkoulis, Fabian, Frost, Luca, Frulloni, Phil, A Hart, Gabriela, Hernandez-Molina, Dai, Inoue, Karuna, Keat, Terumi, Kamisawa, Shigeyuki, Kawa, Mitsuhiro, Kawano, Arezou, Khosroshahi, Hiroshi, Kobayashi, Yuzo, Kodama, Satoshi, Kubo, Kensuke, Kubota, Marco, Lanzillotta, Markus, M Lerch, Yanying, Liu, Matthias, Löhr, Chiara, Marvisi, Ferran, Martinez-Valle, Eduardo, Martin-Nares, Yasufumi, Masaki, Shoko, Matsui, Ichiro, Mizushima, Ray, P Naden, Seiji, Nakamura, Jan, Nordeide, Kenji, Notohara, Kazuichi, Okazaki, Sergio, Paira, Cory, A Perugino, Jovan, Popovic, Manel, Ramos-Casals, James, Rosenbaum, Jay, Ryu, Yasuharu, Sato, Amita, Sharma, Takako, Saeki, Hiroshi, Sekiguchi, Nicolas, Schleinitz, Evgeniya, V Sokol, John, H Stone, James, R Stone, Hiroki, Takahashi, Naoki, Takahashi, Masayuki, Takahira, Yoshiya, Tanaka, Hisanori, Umehara, Vaglio, Augusto, Alejandra, Villamil, Yoko, Wada, Zachary, S Wallace, George, Webster, Kazunori, Yamada, Motohisa, Yamamoto, Joanne, Yi, Giuseppe, Zamboni, Yoh, Zen, Wen, Zhang
Přispěvatelé: Wallace, Z, Zhang, Y, Perugino, Ca, Naden, R, Choi, Hk, Stone, Jh, for the ACR/EULAR IgG4-RD Classification Criteria, Committee, DELLA TORRE, E
Jazyk: angličtina
Rok vydání: 2019
Předmět:
Male
IgG4-related disease
cluster analysis
epidemiology
Adult
Americas
Aortitis
Asia
Asian Continental Ancestry Group
Continental Population Groups
Cross-Sectional Studies
Digestive System Diseases
Europe
Female
Humans
Immunoglobulin G
Immunoglobulin G4-Related Disease
Middle Aged
Mikulicz' Disease
Otorhinolaryngologic Diseases
Phenotype
Retroperitoneal Fibrosis
Disease
Retroperitoneal fibrosis
0302 clinical medicine
Epidemiology
Immunology and Allergy
030212 general & internal medicine
Latent class model
Cohort
medicine.symptom
medicine.medical_specialty
Immunology
General Biochemistry
Genetics and Molecular Biology

Article
03 medical and health sciences
Rheumatology
Asian People
Internal medicine
medicine
030203 arthritis & rheumatology
business.industry
Racial Groups
medicine.disease
Etiology
business
Zdroj: Annals of the Rheumatic Diseases. 78(3)
ISSN: 1468-2060
0003-4967
Popis: ObjectiveIgG4-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.MethodsWe 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.ResultsIn 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, pConclusionWe 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.
Databáze: OpenAIRE