Expert review of clinical immunology · 2026
Measuring disease activity in juvenile systemic sclerosis: a multidisciplinary consensus from the Hamburg 2024 symposium.
Consensus StatementСтатьяОбзорПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод в очереди
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Foeldvari Ivan, Torok Kathryn S, Klotsche Jens, Li Jonathan C, Bruni Cosimo, Costa-Reis Patricia, Cutolo Maurizio, Denton Christopher P, Fligelstone Kim, Del Galdo Francesco, Giménez-Roca Clara, Henes Jörg, Kasapcopur Ozgur, Li Suzanne C, Marrani Edoardo, Orteu Catherine H, Pauling John D, Pilkington Clarissa, Ross Laura, Schonenberg-Meinema Dieneke, Furst Daniel E, Smith Vanessa
Аннотация
INTRODUCTION: Assessment of disease activity in juvenile systemic sclerosis (jSSc) is essential for clinical care and trial readiness, yet no validated pediatric activity measures exist. Adult systemic sclerosis activity tools, including the Scleroderma Clinical Trials Consortium Activity Index, revised CRISS, and revised EUSTAR, provide conceptual frameworks but require adaptation for developmental, physiologic, and feasibility considerations for children. At the 17th Hamburg Symposium on JSSc, an international multidisciplinary panel reviewed adult indices, evaluated corresponding variables in the jSSc Inception Cohort and the NRCOS registry, and conducted a structured Delphi process to define organ-specific indicators of clinically meaningful activity in jSSc.
AREAS COVERED: Across skin, pulmonary, cardiac, vascular, musculoskeletal, gastrointestinal, renal, and global domains, the panel reached broad and often unanimous consensus on variables reflecting active, potentially reversible disease. Key endorsed measures included mRSS progression, new ILD on HRCT, ≥10% declines in FVC or DLCO, new cardiac abnormalities, active digital ulcers, synovitis, myositis, nutritional decline, and physician global assessment. Patient-reported outcomes were strongly supported across domains.
EXPERT OPINION: These consensus-derived indicators provide the first comprehensive pediatric-specific foundation for defining disease activity in jSSc and represent a critical step toward developing and validating a unified pediatric activity index suitable for future clinical trials.
AREAS COVERED: Across skin, pulmonary, cardiac, vascular, musculoskeletal, gastrointestinal, renal, and global domains, the panel reached broad and often unanimous consensus on variables reflecting active, potentially reversible disease. Key endorsed measures included mRSS progression, new ILD on HRCT, ≥10% declines in FVC or DLCO, new cardiac abnormalities, active digital ulcers, synovitis, myositis, nutritional decline, and physician global assessment. Patient-reported outcomes were strongly supported across domains.
EXPERT OPINION: These consensus-derived indicators provide the first comprehensive pediatric-specific foundation for defining disease activity in jSSc and represent a critical step toward developing and validating a unified pediatric activity index suitable for future clinical trials.