Jordi Antón, Juan Manuel Mosquera, Joan Calzada, Estíbaliz Iglesias, Andrea Zacarías, Alejandro Olivé, Violeta Bittermann, Tania Rodríguez Lorenzo, Agustín Remesal, Cristian Quintana-Ortega, Laura Nuño-Nuño, Angel Robles-Marhuenda, Jaime de Inocencio, María Martín-López, Patricia E Carreira, Anahy M Brandy-García, Susana Holgado, Marisol Camacho-Lovillo, Alberto Ruiz-Román, Daniel Clemente, Javier Narváez, José Campos, Judith Sánchez-Manubens, Pilar Bernabéu, Jenaro Graña, Carmen Vargas, Vera Ortiz-Santamaria, Santos Castañeda, María Jesús García de Yébenes, Loreto Carmona
{"title":"Similarities and differences between systemic juvenile idiopathic arthritis and adult-onset Still's disease: a multicenter Spanish study.","authors":"Jordi Antón, Juan Manuel Mosquera, Joan Calzada, Estíbaliz Iglesias, Andrea Zacarías, Alejandro Olivé, Violeta Bittermann, Tania Rodríguez Lorenzo, Agustín Remesal, Cristian Quintana-Ortega, Laura Nuño-Nuño, Angel Robles-Marhuenda, Jaime de Inocencio, María Martín-López, Patricia E Carreira, Anahy M Brandy-García, Susana Holgado, Marisol Camacho-Lovillo, Alberto Ruiz-Román, Daniel Clemente, Javier Narváez, José Campos, Judith Sánchez-Manubens, Pilar Bernabéu, Jenaro Graña, Carmen Vargas, Vera Ortiz-Santamaria, Santos Castañeda, María Jesús García de Yébenes, Loreto Carmona","doi":"10.1007/s00296-024-05658-6","DOIUrl":null,"url":null,"abstract":"<p><p>To describe the characteristics of systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD), compare their presentation and evolution, and analyse possible complication predictors. Multicenter study. Data were retrieved from a hospital-based study of patients with a diagnosis or suspected diagnosis of sJIA or AOSD according to the responsible physician and followed-up for at least one year. Descriptive variables (classification criteria, clinical manifestations, complications, family, and personal history) were collected at disease onset and during follow-up. We present the clinical characteristics of 326 patients, 67% of whom had a diagnosis of sJIA and 33% of AOSD. Clinical manifestation frequencies were similar between the two groups, except for odynophagia, which was significantly more frequent in AOSD than in sJIA (78.4% vs. 25.5%; p < 0.0001). Among the complications, macrophage activation syndrome (MAS) was significantly more common in sJIA than in AOSD (24.4% vs. 9.5%; p = 0.002), to the extent that an sJIA diagnosis significantly increased the risk of MAS, together with serositis presence, and the need for biological therapy. Patients with sJIA and AOSD showed similar characteristics, supporting the idea that they are both part of Still's disease, but are expressed at different ages. Differences in manifestations and complications might be due to different management between diseases and immune response maturity.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":" ","pages":"2911-2920"},"PeriodicalIF":3.2000,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11618169/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Rheumatology International","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1007/s00296-024-05658-6","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/9/23 0:00:00","PubModel":"Epub","JCR":"Q2","JCRName":"RHEUMATOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
To describe the characteristics of systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD), compare their presentation and evolution, and analyse possible complication predictors. Multicenter study. Data were retrieved from a hospital-based study of patients with a diagnosis or suspected diagnosis of sJIA or AOSD according to the responsible physician and followed-up for at least one year. Descriptive variables (classification criteria, clinical manifestations, complications, family, and personal history) were collected at disease onset and during follow-up. We present the clinical characteristics of 326 patients, 67% of whom had a diagnosis of sJIA and 33% of AOSD. Clinical manifestation frequencies were similar between the two groups, except for odynophagia, which was significantly more frequent in AOSD than in sJIA (78.4% vs. 25.5%; p < 0.0001). Among the complications, macrophage activation syndrome (MAS) was significantly more common in sJIA than in AOSD (24.4% vs. 9.5%; p = 0.002), to the extent that an sJIA diagnosis significantly increased the risk of MAS, together with serositis presence, and the need for biological therapy. Patients with sJIA and AOSD showed similar characteristics, supporting the idea that they are both part of Still's disease, but are expressed at different ages. Differences in manifestations and complications might be due to different management between diseases and immune response maturity.
期刊介绍:
RHEUMATOLOGY INTERNATIONAL is an independent journal reflecting world-wide progress in the research, diagnosis and treatment of the various rheumatic diseases. It is designed to serve researchers and clinicians in the field of rheumatology.
RHEUMATOLOGY INTERNATIONAL will cover all modern trends in clinical research as well as in the management of rheumatic diseases. Special emphasis will be given to public health issues related to rheumatic diseases, applying rheumatology research to clinical practice, epidemiology of rheumatic diseases, diagnostic tests for rheumatic diseases, patient reported outcomes (PROs) in rheumatology and evidence on education of rheumatology. Contributions to these topics will appear in the form of original publications, short communications, editorials, and reviews. "Letters to the editor" will be welcome as an enhancement to discussion. Basic science research, including in vitro or animal studies, is discouraged to submit, as we will only review studies on humans with an epidemological or clinical perspective. Case reports without a proper review of the literatura (Case-based Reviews) will not be published. Every effort will be made to ensure speed of publication while maintaining a high standard of contents and production.
Manuscripts submitted for publication must contain a statement to the effect that all human studies have been reviewed by the appropriate ethics committee and have therefore been performed in accordance with the ethical standards laid down in an appropriate version of the 1964 Declaration of Helsinki. It should also be stated clearly in the text that all persons gave their informed consent prior to their inclusion in the study. Details that might disclose the identity of the subjects under study should be omitted.