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Войти в исследования Age and onset timing of Raynaud's phenomenon and first non-Raynaud symptom as prognostic factors in systemic sclerosis: a retrospective analysis from the Italian national multicenter Systemic Sclerosis Progression INvestiGation registry of the Italian Society for Rheumatology (SPRING-SIR).
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Peretti Silvia, Bruni Cosimo, Bonomi Francesco, De Angelis Rossella, Bajocchi Gianluigi, Giuggioli Dilia, Orlandi Martina, Zanframundo Giovanni, Foti Roberta, Visalli Elisa, Cuomo Giovanna, Ariani Alarico, Rosato Edoardo, Lepri Gemma, Girelli Francesco, Riccieri Valeria, Zanatta Elisabetta, Bosello Silvia Laura, Cavazzana Ilaria, Ingegnoli Francesca, De Santis Maria, Cacciapaglia Fabio, Murdaca Giuseppe, Abignano Giuseppina, Pettiti Giorgio, Della Rossa Alessandra, Caminiti Maurizio, Iuliano Annamaria, Ciano Giovanni, Beretta Lorenzo, Bagnato Gianluca, Lubrano Ennio, De Andres Ilenia, Idolazzi Luca, Saracco Marta, Agnes Cecilia, Campochiaro Corrado, De Luca Giacomo, Cipolletta Edoardo, Fornaro Marco, Lumetti Federica, Spinella Amelia, Magnani Luca, Codullo Veronica, Iandoli Carlo, Gigante Antonietta, Pellegrino Greta, Pigatto Erika, Lazzaroni Maria Grazia, De Lorenzis Enrico, Mennillo Gianna Angela, Battista Marco Di, Pagano Mariano Giuseppa, Furini Federica, Vultaggio Licia, Parisi Simone, Peroni Clara Lisa, Bianchi Gerolamo, Fusaro Enrico, Sebastiani Gian Domenico, Govoni Marcello, D'Angelo Salvatore, Cozzi Franco, Franceschini Franco, Guiducci Serena, Dagna Lorenzo, Doria Andrea, Salvarani Carlo, D'Agostino Maria Antonietta, Iannone Florenzo, Matucci-Cerinic Marco, Ferri Clodoveo, Randone Silvia Bellando
Аннотация
OBJECTIVES: To evaluate whether the mode and ages of clinical onset are associated with disease endotype and survival in SSc.
DESIGN: We included SSc patients from the Systemic sclerosis Progression INvestiGation registry of the Italian Society of Rheumatology (SPRING-SIR) registry in a cohort study, with post hoc cross-sectional and longitudinal analysis.
METHODS: Patients were grouped based on age-RP and age-NRP quartiles. Additionally, categories were defined based on mode of onset: RP group-RP onset at least 1 year before NRP; Simultaneous group-RP onset within the same year of NRP; NRP group-RP onset after at least 1 year after NRP. Comparisons were made using Chi-square and ANOVA tests. Logistic, linear, and multinomial regression models were applied to assess associations, while Kaplan-Meier curves and Cox regression were used to assess mortality.
RESULTS: A total of 1748 patients were eligible: 682 (39.0%) in the RP group, 1026 (58.8%) in the simultaneous group, and 39 (2.2%) in the NRP group. A higher prevalence of anti-centromere antibodies was found In the RP group, while the simultaneous group had more diffuse cutaneous SSc (dcSSc), anti-topoisomerase-I antibodies, and higher Rodnan's skin score (mRSS). The NRP group presented higher prevalence of pulmonary arterial hypertension. On logistic regression, the simultaneous group was associated with a higher prevalence of dcSSc compared to the RP group (odds ratio, 1.491, 95% confidence interval (CI): 1.032-2.154). Younger age at RP onset was associated with lower systolic pulmonary artery pressure and mRSS. In 943 patients with available follow-up (median 24 months), the simultaneous group had higher mortality compared to the RP group (hazard ratio, 1.975, 95% CI: 1.002-3.893).
CONCLUSION: The timing of RP and NRP onset may help define SSc endotype and survival. Patients with simultaneous RP-NRP onset have more severe disease features and higher mortality risk, emphasizing the relevance of onset timing in disease stratification.