FRI0230 THE 2009-2019 SURVIVAL AND MORTALITY PREDICTORS IN A LARGE MULTICENTRE SYSTEMIC SCLEROSIS COHORT
Authors: F. Cacciapaglia, E. De Lorenzis, A. Corrado, S. L. Bosello, M. Fornaro, F. Montini, L. Urso, L. Verardi, A. Altomare, F. P. Cantatore, E. Gremese, F. Iannone
Journal: Annals of the Rheumatic Diseases (2020)

Background: Systemic sclerosis (SSc) is one of the connective tissue diseases with the poorer prognosis and disease-related causes, particularly pulmonary fibrosis, PAH and cardiac involvement, accounting the most deaths. Objectives: This multicentre study aimed to evaluate the global survival and any predictor of mortality in a large multicentric cohort of SSc patients. Methods: We performed a retrospective analysis examining the medical records of our longitudinal SSc cohorts with a median (IQR) follow-up of 11 (6-18) years from 3 Scleroderma Units since January 2009. All clinical, laboratory and instrumental findings have been recorded and analyzed using Chi-squared tests, Kaplan-Meier curves, log-rank tests, and Cox proportional hazards models. Results: Data from 750 SSc patients (91.9% female; mean (SD) age at first Non-Raynaud symptom 48.4 (15.3) years, median (IQR) disease duration 3 (0-8) years; diffuse cutaneous involvement 162 (21.6%) patients) fulfilling the 1980 ARA and/or 2013 ACR/EULAR classification criteria, were collected. All patients were positive for ANA, anti-Topo-I Abs were found in 235 (31.3%) and Cenp-B Abs in 300 (40%) patients. 98 (13.1%) patients were positive to other Abs (Anti-RNA polymerase III, anti-Pm/Scl) and anti-ENA were negative/unknown for 117 (15.6%) patients. Interstitial lung disease (ILD) was present in 202 (26.9%), pulmonary arterial hypertension (PAH) was found in 29 (3.9%), and 50/750 (6.7%) patients presented pulmonary hypertension combined with ILD (PH-ILD). The overall 10-years survival was 93.1% and, it was significantly impaired by the presence of ILD, PAH or PH-ILD [Figure]. The univariate analysis showed that female gender, higher age at first Non-Raynaud symptom, earlier referral to a tertiary Scleroderma center, absence of any ENA antibodies, and PH-ILD presence were survival predictors. After multivariate analysis the significance of PH-ILD was lost [Table]. Disease duration, basal Rodnan skin score, smoking, renal or gastrointestinal comorbidities, NYHA functional class, steroid or immune-suppressive treatments did not reach the statistically significance. Conclusion: Our study demonstrated a global 10-years survival rate over 93%. Male patients and rapid evolution of Non-Raynaud symptoms represent the main death predictors in our SSc cohort. A rapid referral to a tertiary rheumatological centre and early treatment with effective agents are associated to a better prognosis. Figure. Kaplan-Meier curves for 5-years survival in SSc patients (Log-rank 8.96, p=0.03). Table. Prognostic factors for 10-years survival at univariate and multivariate analysis. UNIVARIATE ANALYSIS MULTIVARIATE ANALYSIS HR 95%IC P HR 95%IC P Female gender 0.35 0.15-0.81 0.01 0.31 0.15-0.66 0.002 Age at first Non-Raynaud symptom 1.07 1.04-1.1 0.001 1.08 1.05-1.11 0.001 Time referral to a tertiary SSc centre 0.83 0.76-0.92 0.001 0.84 0.77-0.93 0.001 Absence of any ENA antibodies 0.08 0.01-0.62 0.01 0.09 0.01-0.71 0.02 PH-ILD presence 2.6 1.01-6.82 0.04 2.4 0.93-6.1 0.069 Disclosure of Interests: Fabio Cacciapaglia Speakers bureau: BMS; Roche; Pfizer; Abbvie, Enrico De Lorenzis: None declared, Addolorata Corrado: None declared, Silvia Laura Bosello Speakers bureau: Abbvie, Pfizer, Boehringer, Marco Fornaro: None declared, Fabio Montini: None declared, Livio Urso: None declared, Lucrezia Verardi: None declared, Alberto Altomare: None declared, Francesco Paolo Cantatore: None declared, Elisa Gremese Consultant of: AbbVie, Bristol-Myers Squibb, Celgene, Eli Lilly, Janssen, Merck Sharp &amp; Dohme, Novartis, Sanofi, UCB, Roche, Pfizer, Speakers bureau: AbbVie, Bristol-Myers Squibb, Celgene, Eli Lilly, Janssen, Merck Sharp &amp; Dohme, Novartis, Sanofi, UCB, Roche, Pfizer, Florenzo Iannone Consultant of: Speaker and consulting fees from AbbVie, Eli Lilly, Novartis, Pfizer, Roche, Sanofi, UCB, MSD, Speakers bureau: Speaker and consulting fees from AbbVie, Eli Lilly, Novartis, Pfizer, Roche, Sanofi, UCB, MSD