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 with SSc showed a fivefold increased mortality, with a trend toward a higher risk in young-onset (SMR 6.3; 95 %CI 4.1–9.1) compared with elderly-onset (SMR 4.5; 95 %CI 2.4–7.7) cases. Conclusions: Elderly-onset identifies a distinct clinical subset of SSc, mainly characterized by mild cutaneous and peripheral vascular involvement, but showing a greater burden of pulmonary vascular disease and increased mortality compared to the age-matched general population.
Elderly-onset systemic sclerosis defines a distinct clinical subset: analysis from the SPRING registry of the Italian Society for Rheumatology / A. Tonutti, F. Motta, G. Bajocchi, S. Bellando-Randone, C. Bruni, M. Orlandi, G. Zanframundo, R. Foti, G. Cuomo, A. Ariani, E. Rosato, G. Lepri, F. Girelli, E. Zanatta, S.L. Bosello, I. Cavazzana, F. Ingegnoli, F. Cacciapaglia, G. Murdaca, G. Abignano, G. Pettiti, A. Della Rossa, M. Caminiti, A. Iuliano, G. Ciano, L. Beretta, G. Bagnato, E. Lubrano, I. 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.G. Lazzaroni, E. De Lorenzis, G. Mennillo, M. Di Battista, G. Pagano-Mariano, F. Furini, L. Vultaggio, S. Parisi, M.C. Ditto, G. Bianchi, E. Fusaro, G.D. Sebastiani, M. Govoni, S. D'Angelo, F. 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. - In: JOURNAL OF AUTOIMMUNITY. - ISSN 0896-8411. - 157:(2025), pp. 103501.1-103501.11. [10.1016/j.jaut.2025.103501]
Elderly-onset systemic sclerosis defines a distinct clinical subset: analysis from the SPRING registry of the Italian Society for Rheumatology
F. Ingegnoli;G. Pellegrino;
2025
Abstract
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 with SSc showed a fivefold increased mortality, with a trend toward a higher risk in young-onset (SMR 6.3; 95 %CI 4.1–9.1) compared with elderly-onset (SMR 4.5; 95 %CI 2.4–7.7) cases. Conclusions: Elderly-onset identifies a distinct clinical subset of SSc, mainly characterized by mild cutaneous and peripheral vascular involvement, but showing a greater burden of pulmonary vascular disease and increased mortality compared to the age-matched general population.
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