AB0640 SURVIVAL AND MORTALITY PREDICTORS IN SYSTEMIC SCLEROSIS: RESULTS FROM A MONOCENTRIC ITALIAN COHORT
Authors: Fabio Cacciapaglia, Marco Fornaro, Fabio Montini, Livio Urso, Sergio Colella, Laura Coladonato, Maria Grazia Anelli, Emanuela Praino, Giuseppe Lopalco, Florenzo Iannone
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background:</h3> Systemic sclerosis (SSc) is a connective tissue disease with a poor prognosis and disease-related pulmonary fibrosis, pulmonary arterial hypertension (PAH) and cardiac involvement accounted for most deaths (1). However, a decrease in mortality is seen in the last decades thanks to the opportunity of new screening and therapeutic strategies (2). <h3>Objectives:</h3> To estimate the global survival and any predictor of mortality in a monocentric cohort of SSc patients with a 10-years follow-up. <h3>Methods:</h3> We performed a retrospective analysis examining the medical records of our longitudinal SSc cohort with a median (IQR) follow-up of 56 (25-82.5) months in our Scleroderma Unit 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 modeling. <h3>Results:</h3> We collected data from 347 SSc patients (female n. 319 (91.9%); mean (SD) age at diagnosis 50.6 (15.9) years, median (IQR) disease duration 8.75 (4.1-14.9) years; diffuse cutaneous involvement (dsSSc) n.57 (16.4%) patients) fulfilling the 1980 aRA and/or 2013 aCR/EULAR classification criteria. All patients were positive for aNA, while anti-Topo-I were found in 145 (41.8%), CENP-B in 139 (40.1%) patients. Fifteen (4.3%) patients were positive to other autoantibodies (Anti-RNA polymerase III, anti-Pm/Scl) while anti-ENA were negative or unknown for 48 (13.8%) SSc patients. Interstitial lung disease (ILD) was present in 158 (45.5%), pulmonary arterial hypertension, diagnosed by right heart catheterism, (Group 1 PAH) was found in 17 (4.9%), and 22/347 (6.3%) patients presented pulmonary hypertension combined with ILD (Group 3 PH-ILD). The overall survival rates were 89.1% and 87.3% at 5 and 10 years, respectively. As reported in Figure 1, the 5-years global survival was significantly impaired by the presence of PAH or PH-ILD, while isolated ILD did not impact the survival. The univariate and multivariate analysis showed that dsSSc, higher age at diagnosis, delayed referral to our Scleroderma Unit, body mass index and absence of heart (H) or lung (L) involvement (No H/L) were independent predictors of 5-years mortality (Table I). While gender, disease duration, specific autoantibodies, basal Rodnan skin score, smoking, renal or gastrointestinal comorbidities, NYHA functional class, steroid or immune-suppressive treatments did not reach the statistically significance. <h3>Conclusion:</h3> Our study demonstrated a global 10-years survival rate over 85%, and the presence of PH with or without ILD represents the main negative predictor. The rapid referral to a specialized centre for SSc and early treatment with effective agents for PH could further improve prognosis. <h3>References</h3> [1] Tyndall aJ, et al. Ann Rheum Dis. 2010. [2] allanore Y, et al. Expert Opin Pharmacother. 2007. <h3>Disclosure of interests:</h3> Fabio Cacciapaglia: None declared, Marco fornaro: None declared, Fabio Montini: None declared, Livio Urso: None declared, Sergio Colella: None declared, Laura Coladonato: None declared, Maria Grazia anelli: None declared, Emanuela Praino: None declared, Giuseppe Lopalco Speakers bureau: SOBI, BMS, Florenzo Iannone Consultant for: F Iannone has received consultancy fees and/or speaker honoraria from Pfizer, abbVie, MSD, BMS, Novartis, Lilly, UCB outside this work, Speakers bureau: F Iannone has received consultancy fees and/or speaker honoraria from Pfizer, abbVie, MSD, BMS, Novartis, Lilly, UCB outside this work