AB1213 FIVE-YEAR SURVIVAL AND PROGNOSTIC PREDICTORS IN PATIENTS WITH SYSTEMIC SCLEROSIS ASSOCIATED PULMONARY ARTERIAL HYPERTENSION
Authors: S. Stano, C. Iannone, A. Capuano, M. Lavista, M. Morrone, A. Cito, C. Ammoscato, N. Del Papa, R. F. Caporali, F. Cacciapaglia
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Pulmonary arterial hypertension (PAH) can complicate systemic sclerosis (SSc), representing the leading cause of death in these patients. According to recent epidemiological evidence, about 10% of SSc patients may be affected by PAH [1], with a mortality rate of about 50% after 3 years from PAH diagnosis [2]. Few and outdated information on SSc-PAH in the Italian population is available, furthermore it would be advisable to look at any new prognostic factors for improving the outcome. <h3>Objectives:</h3> This study aimed to assess the updated 5-year survival rate and prognostic predictors in a multicenter Italian SSc-PAH cohort. <h3>Methods:</h3> SSc patients, fulfilling the 2013 EULAR/ACR classification criteria [3], complicated by PAH at right heart catheterization (RHC), according to the updated 2022 ESC/ERS guidelines for PH [4], were included in this study. Demographic, clinical, laboratory and instrumental data of SSc-PAH patients, visited until December 2023, were recorded. All data were analyzed using SPSS with appropriate statistical tests. The survival rate was assessed using the Kaplan-Meier method, while predictors of mortality were expressed as odds ratios (OR) and 95% Confidence Intervals (CI). <h3>Results:</h3> Fifty-one SSc-PAH patients, with a median follow-up time of 4.0 years since RHC diagnosis, were identified [Table 1]. The 5-years survival rate in our cohort of SSc-PAH patients was 66.7% [Figure 1]. SSc patients with a worse WHO functional class (NYHA III-IV) and with a 4-strata class of risk intermediate-high/high at PAH diagnosis had a trend towards lower survival probability, but statistical significance was not achieved. Notably, SSc patients with poorer prognoses had a significantly lower % of predicted FVC at PAH diagnosis (p&lt;0.01), and predicted FVC &lt;70% (OR 9.75 95%CI 1.7-51.2 – p=0.01) resulted to be a statistically significant poor survival predictor. Moreover, a steeper decrease from PAH diagnosis in the predicted % of FVC and % of DLCO (p&lt;0.05) was associated with a poor prognosis, while the baseline or last follow-up visit values of predicted % of DLCO were not significantly different between survived and deceased patients. None of the examined characteristics including age, gender, disease duration, skin or gastrointestinal involvement, autoantibody profile, echocardiographic parameters, or initial therapeutic strategy in monotherapy or combination, achieved a statistically significant impact on survival. Of importance, all SSc patients were promptly upgraded to appropriate PAH therapy during follow-up, and after 3 years all SSc patients were at least in combination therapy. <h3>Conclusion:</h3> Effective screening and early diagnosis of PAH, with close clinical monitoring and timely and effective therapeutic adjustments, have a positive impact on the survival of patients with SSc. A low %FVC predicted and a progressive decrease in % DLCO predicted are the main negative prognostic factors. Furthermore, a careful mortality risk assessment in SSc-PAH patients, using the recommended risk stratification tools (i.e. COMPERA 2.0), is easily implemented and effective in clinical practice. <h3>REFERENCES:</h3> [1] Rubio-Rivas M et al. Autoimmunity Reviews. 2021;20(1):102713. [2] Distler O et al. Rheumatology. Published online July 18, 2023:kead360. [3] van den Hoogen F et al. Ann Rheum Dis. 2013;72(11):1747-1755. [4] Humbert M et al. European Heart Journal. 2022;43(38):3618-3731. <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>