Similarities and differences between systemic juvenile idiopathic arthritis and adult-onset Still’s disease: a multicenter Spanish study

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.

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Publication Details

Journal
Rheumatology International
Published
2024-09-23
DOI
https://doi.org/10.1007/s00296-024-05658-6
Citations
2
Primary Topic
Autoimmune and Inflammatory Disorders Research
Type
article
Field-Weighted Citation Impact
0.91

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article

Similarities and differences between systemic juvenile idiopathic arthritis and adult-onset Still’s disease: a multicenter Spanish study

Loreto Carmona, María Jesús García de Yébenes, Joan Calzada‐Hernández, Vera Ortiz-Santamaría et al.
2 citations
Rheumatology International
Autoimmune and Inflammatory Disorders Research
0.91
article

Similarities and differences between systemic juvenile idiopathic arthritis and adult-onset Still’s disease: a multicenter Spanish study

Loreto Carmona, María Jesús García de Yébenes, Joan Calzada‐Hernández, Vera Ortiz-Santamaría, Javier Narváez, Juan Manuel Mosquera, Santos Castañeda, C Maguiña Vargas, Marisol Camacho‐Lovillo, Pilar Bernabeu Gonzálvez, María Martín-López, Jordi Antón, Andrea Zacarías, E. Iglesias, Ángel Robles‐Marhuenda, Judith Sánchez-Manubens, Violeta Bittermann, Daniel Clemente, Cristian Quintana-Ortega, Susana Holgado Pérez, Patricia Esmeralda Carreira, Jaime de Inocencio Arocena, Agustín Remesal, Anahy Brandy-García, J. García Campos, Alberto Ruíz-Román, T. Lorenzo, Jenaro Graña Gil, Laura Nuño, Alejandro Olivé
article en
2 citations

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.

Rheumatology InternationalVol. 44(12)
Hospital Sant Joan de Déu Barcelona (ES), Hospital Universitario La Paz (ES), Bellvitge University Hospital (ES), Hospital Universitario Virgen Macarena (ES), Hospital Universitario 12 De Octubre (ES), Hospital Universitario Puerta de Hierro Majadahonda (ES), Hospital General de Granollers (ES), Hospital Infantil Universitario Niño Jesús (ES), Servicio Canario de la Salud (ES), Hospital Universitario de La Princesa (ES), Hospital General Universitario de Alicante Doctor Balmis (ES), Complexo Hospitalario Universitario A Coruña (ES), Hospital Universitario Virgen del Rocío (ES), Hospital Universitari Germans Trias i Pujol (ES), Instituto de Salud Musculoesquelética (ES), Universitat de Barcelona (ES)
Universitat de Barcelona
Openalex Percentile: Top 23%
Autoimmune and Inflammatory Disorders Research
0.91
DOIOpen Access PDFOpenAlex
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