Elderly-onset systemic sclerosis defines a distinct clinical subset: analysis from the SPRING registry of the Italian Society for Rheumatology.

作者：A. Tonutti, F. Motta, G. Bajocchi, S. Bellando-Randone, Cosimo Bruni, M. Orlandi, G. Zanframundo, R. Foti, G. Cuomo, A. Ariani, E. Rosato, Gemma Lepri, F. Girelli, E. Zanatta, S. Bosello, I. Cavazzana, F. Ingegnoli, F. Cacciapaglia, G. Murdaca, G. Abignano, G. Pettiti, A. Della Rossa, Maurizio Caminiti, A. Iuliano, G. Ciano, Lorenzo Beretta, G. Bagnato, E. Lubrano, Ilenia de Andres, L. Idolazzi, M. Saracco, C. Agnes, C. Campochiaro, M. Nivuori, E. Cipolletta, F. Lumetti, A. Spinella, E. Cocchiara, G. de Luca, V. Codullo, E. Visalli, C. Iandoli, A. Gigante, G. Pellegrino, E. Pigatto, M. Lazzaroni, E. De Lorenzis, G. Mennillo, M. Di Battista, G. Pagano-Mariano, F. Furini, Licia Vultaggio, Simone Parisi, M. C. Ditto, Gerolamo Bianchi, E. Fusaro, G. Sebastiani, M. Govoni, Salvatore D’Angelo, Franco Cozzi, F. Franceschini, S. Guiducci, L. Dagna, A. Doria, D. Giuggioli, V. Riccieri, C. Salvarani, F. Iannone, C. Ferri, M. Matucci-Cerinic, M. De Santis, R. De Angelis · 发表于：Journal of Autoimmunity · 年份：2025 · DOI：10.1016/j.jaut.2025.103501 · 被引用次数：4 · 研究领域：Medicine

OBJECTIVE Elderly-onset systemic sclerosis (SSc) is relatively uncommon, and its clinical phenotype and prognostic implications remain poorly characterized, with conflicting evidence regarding disease course and outcomes. METHODS Within the Italian SPRING (Systemic Sclerosis PRogression INvestiGation) registry, we compared demographic and clinical characteristics of patients with elderly-onset SSc (≥70 years at the time of the first non-Raynaud's manifestation) to those with younger onset. Cross-sectional analyses, multivariable logistic regression, and unsupervised cluster analysis were conducted to identify features associated with elderly-onset SSc. Longitudinal analysis was performed to assess mortality risk within SSc patients and compared to the general Italian population. RESULTS Elderly-onset accounted for 8.5 % (160/1893) SSc cases in SPRING. These patients exhibited fewer peripheral vascular complications (digital ulcers: 13 % vs. 23 %; p = 0.016), higher prevalence of anticentromere antibodies (60 % vs. 39 %; p = 0.007), a lower prevalence and likelihood of diffuse skin subset (OR 0.40; 95 % CI 0.19-0.83) but an increased risk of pulmonary arterial hypertension confirmed on right-heart catheterization (OR 14.1; 95 % CI 3.68-54.5) at multivariate analysis. As expected, patients with elderly onset SSc had an increased risk of death compared to younger-onset individuals. Compared with the age-, sex-, and calendar year-matched general Italian population, patients w...