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FRI0230 (2020)
THE 2009-2019 SURVIVAL AND MORTALITY PREDICTORS IN A LARGE MULTICENTRE SYSTEMIC SCLEROSIS COHORT
F. Cacciapaglia1, E. De Lorenzis2, A. Corrado3, S. L. Bosello2, M. Fornaro1, F. Montini1, L. Urso1, L. Verardi2, A. Altomare3, F. P. Cantatore3, E. Gremese2, F. Iannone1
1Rheumatology Unit, Department of Emergency and Organ Transplantations - University and AOU Policlinico of Bari, Bari, Italy
2Division of Rheumatology, Fondazione Policlinico Universitario A. Gemelli IRCCS, Università Cattolica del Sacro CuoreFondazione Policlinico Universitario A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, Rome, Italy
3Rheumatology Clinic, Department of Medical and Surgical Sciences - University of Foggia, Foggia, Italy

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.

Kaplan-Meier curves for 5-years survival in SSc patients (Log-rank 8.96, p=0.03).

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 & Dohme, Novartis, Sanofi, UCB, Roche, Pfizer, Speakers bureau: AbbVie, Bristol-Myers Squibb, Celgene, Eli Lilly, Janssen, Merck Sharp & 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

Citation: Ann Rheum Dis, volume 79, supplement 1, year 2020, page 694
Session: Scleroderma, myositis and related syndromes (Poster Presentations)