bjective: 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 / Tonutti, Antonio; Motta, Francesca; Bajocchi, Gianluigi; Bellando-Randone, Silvia; Bruni, Cosimo; Orlandi, Martina; Zanframundo, Giovanni; Foti, Roberta; Cuomo, Giovanna; Ariani, Alarico; Rosato, Edoardo; Lepri, Gemma; Girelli, Francesco; Zanatta, Elisabetta; Laura Bosello, Silvia; Cavazzana, Ilaria; Ingegnoli, Francesca; Cacciapaglia, Fabio; Murdaca, Giuseppe; Abignano, Giuseppina; Pettiti, Giorgio; Della Rossa, Alessandra; Caminiti, Maurizio; Iuliano, Annamaria; Ciano, Giovanni; Beretta, Lorenzo; Bagnatoa, Gianluca; I, Ennio Lubrano; De Andres, Lenia; Idolazzi, Luca; Saracco, Marta; Agnes, Cecilia; Campochiaro, Corrado; Nivuori, Mariangela; Cipolletta, Edoardo; Lumetti, Federica; Spinella, Amelia; Cocchiara, Emanuele; De Luca, Giacomo; Codullo, Veronica; Visalli, Elisa; Iandoli, Carlo; Gigante, Antonietta; Pellegrino Erika Pigatto, Greta; Grazia Lazzaroni, Maria; De Lorenzis, Enrico; Mennillo, Gianna; Di Battista, Marco; Pagano-Mariano, Giuseppa; Furini, Federica; Vultaggio, Licia; Parisi, Simone; Chiara Ditto, Maria; Bianchi, Gerolamo; Fusaro, Enrico; Domenico Sebastiani, Gian; Govoni, Marcello; D’Angelo, Salvatore; Cozzi, Franco; Franceschini, Franco; Guiducci, Serena; Dagna, Lorenzo; Doria, Andrea; Giuggioli, Dilia; Riccieri, Valeria; Salvarani, Carlo; Iannone, Florenzo; Ferri, Clodoveo; Matucci-Cerinic, Marco; De Santis, Maria; De Angelis, Rossella. - In: JOURNAL OF AUTOIMMUNITY. - ISSN 0896-8411. - (2025), pp. 1-11. [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
Martina Orlandi;Federica Lumetti;Amelia Spinella;Dilia Giuggioli;Carlo Salvarani;Clodoveo Ferri;
2025
Abstract
bjective: 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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