AB0432 SURVIVAL AND PROGNOSIS IN SYSTEMIC SCLEROSIS: RESULTS FROM A SINGLE-CENTER COHORT
Authors: M. Rojas-Giménez, R. Ortega Castro, C. López-Medina, J. Calvo Gutierrez, M. Á. Aguirre-Zamorano, A. Escudero Contreras
Journal: Annals of the Rheumatic Diseases (2021)

Objectives: To analyze survival, causes of death, and risk factors associated with mortality in a cohort of patients with Systemic Sclerosis (SSc) at a single center Methods: We performed a retrospective observational study of a cohort of patients with SSc undergoing follow-up during 2012 and until August 2020. We used the Kaplan-Meier method to estimate survival from onset of symptoms and multivariate Cox regression analysis to obtain independent risk factors associated with mortality Results: The study population included 85 patients (women, 85.8%; mean age at diagnosis, 64.4 ± 12.7 years). A total of 19 patients (22.6%) died (table 1). Of these 11 (57.9%) died of a cause related to the disease itself (interstitial lung disease [ILD], 5 (26.3%); pulmonary hypertension, 2 (10.5%); or a combination of both, 3 (15.8%). The main cause of non–SSc-related death was cancer (21.1%). Survival rates at 5, 10, and 20 years were 98%, 92%, and 75%, respectively. Survival was statistically significantly poorer for the absence of ACA, the presence of antitopoisomerase I antibodies, proximal skin thickening, pulmonary hypertension, ILD, cancer and the diffuse subtype. The multivariate analysis performed to determine which factors were independently associated with mortality confirmed that older age at diagnosis of the disease, lower FVC in spirometry at diagnosis of ILD, and proximal skin thickening were associated with greater mortality Table 1. Clinical and immunological characteristics of patients who died and patients who lived Dead (n=19 ) Alive (n=66 ) p -value Female sex, n (%) 17 (89.5) 55 (83.3) 0.594 Age at diagnosis (years), mean (SD) 56.9 (13.7) 50.4 (13.6) 0.076 Time since diagnosis (years), mean (SD) 11.6 (7.3) 14.2 (9.2) 0.215 lcSSc, n (%) 6 (33.3) 50 (75.7) &lt; 0.001 dcSSc, n (%) 12 (66.7) 10 (15.1) &lt; 0.001 Digital ulcers, n (%) 9 (47.4) 33 (50) 0.748 Calcinosis, n (%) 1 (5.3) 16 (24.2) 0.061 Telangiectasias, n (%) 15 (78.9) 56 (84.8) 0.184 ILD, n (%) 15 (78.9) 32 (48.5) 0.021 FEV 1 at diagnosis of ILD, mean (SD) 78.3 (19.3) 78.7 (17.7) 0.955 FVC at diagnosis of ILD, mean (SD) 65.2 (13.1) 78.1 (20.7) 0.043 DLCO at diagnosis of ILD, mean (SD) 56.9 (17.5) 63.9 (16.7) 0.301 Pulmonary hypertension, n (%) 11 (57.9) 14 (21.9) 0.002 sPAP (mmHg), mean (SD) 49.2 (24.7) 30.2 (8.2) 0.009 Gastrointestinal involvement, n (%) 13 (68.4) 30 (45.4) 0.125 Cardiac involvement, n (%) 3 (15.8) 11 (16.7) 0.907 Muscle involvement, n (%) 1 (5.3) 2 (3.03) 0.851 Arthritis or arthralgia, n (%) 5 (26.3) 23 (34.8) 0.436 Renal crisis, n (%) 0 2 (3.03) &lt;0.001 Cancer, n (%) 5 (26.3) 2 (3.03) 0.001 Positive ACA, n (%) 5 (26.3) 35 (53.03) 0.034 Positive ATA, n (%) 10 (52.6) 9 (13.6) &lt; 0.001 Abbreviations: ILD: diffuse interstitial lung disease, SSc: systemic sclerosis, FEV 1 : forced expiratory volume in the first second, FVC: forced vital capacity, DLCO: diffusing capacity for carbon monoxide, sPAP: systolic pulmonary artery pressure, ACA: anticentromere antibody, ATA: antitopoisomerase I antibody. Conclusion: Survival at 10 years was greater than 90% in the study cohort. The main causes of death were ILD, pulmonary hypertension and cancer. The main factors associated with mortality were proximal skin thickening, older age at diagnosis, and lower forced vital capacity Disclosure of Interests: None declared