| Contributors: |
Peretti, S.; Bruni, C.; Bonomi, F.; De Angelis, R.; Bajocchi, G.; Giuggioli, D.; Orlandi, M.; Zanframundo, G.; Foti, R.; Visalli, E.; Cuomo, G.; Ariani, A.; Rosato, E.; Lepri, G.; Girelli, F.; Riccieri, V.; Zanatta, E.; Bosello, S. L.; Cavazzana, I.; Ingegnoli, F.; De Santis, M.; Cacciapaglia, F.; Murdaca, G.; Abignano, G.; Pettiti, G.; Della Rossa, A.; Caminiti, M.; Iuliano, A.; Ciano, G.; Beretta, L.; Bagnato, G.; Lubrano, E.; De Andres, I.; Idolazzi, L.; Saracco, M.; Agnes, C.; Campochiaro, C.; De Luca, G.; Cipolletta, E.; Fornaro, M.; Lumetti, F.; Spinella, A.; Magnani, L.; Codullo, V.; Iandoli, C.; Gigante, A.; Pellegrino, G.; Pigatto, E.; Lazzaroni, M. G.; De Lorenzis, E.; Mennillo, G. A.; Battista, M. D.; Pagano Mariano, G.; Furini, F.; Vultaggio, L.; Parisi, S.; Peroni, C. L.; Bianchi, G.; Fusaro, E.; Sebastiani, G. D.; Govoni, M.; D'Angelo, S.; Cozzi, F.; Franceschini, F.; Guiducci, S.; Dagna, L.; Doria, A.; Salvarani, C.; D'Agostino, M. A.; Iannone, F.; Matucci-Cerinic, M.; Ferri, C.; Randone, S. B. |
| Description: |
Background: The sequence and temporal relationship between Raynaud's phenomenon (RP) and the first non-Raynaud's sign/symptom (NRP) in systemic sclerosis (SSc) have been partially investigated.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. ... |