AB0913 MORTALITY AND ASSOCIATED FACTORS IN 44 PATIENTS WITH SYSTEMIC SCLEROSIS ASSOCIATED PULMONARY ARTERIAL HYPERTENSION FROM TURKEY

Background Pulmonary arterial hypertension (PAH) is a severe complication of systemic sclerosis with high mortality. Although new drugs and treatment strategies developed in the last years, prognosis prevalent patients has not been sufficiently improved. Objectives We aimed to analyze prevalence, mortality, prognostic factors SSc-PAH from our single-center SSc cohort. Methods retrospectively evaluated association between mortality demographics, transthoracic echocardiography, right heart catheterization (RHC) pulmonary function parameters at baseline, modalities that have assessed followed up by multi-disciplinary team. Results Forty-four (10.6%) out 415 were diagnosed as 2008-2022. The mean age was 56.6±13.5 23 (52.3%) deceased during median follow-up 45 months. survival rates 91% for first year, 75% 2 68% 3 43.1% 5 years. available causes death cardiopulmonary failure 6 (26%), infection (26.1%) including one COVİD-19, malignancy (13%) patients. Clinical hemodynamic findings given Table 1. Demographic, serologic, clinical, Demographics (n ) Men Women 42 Characteristics LcSSc 10 DcSSc 34 Digital ulcer 20 Gastrointestinal involvement 27 Arthritis 7 Renal crisis Specific auto-antibodies Anti-centromere 8 Anti-Scl 70 22 Survived Died Patient number (n,% 21 (47.7) (52.3) P-value Nonspecific interstitial pneumonia (NSIP) 0.01 Usual (UIP) Echocardiography ePASP (mmHg) 49.3±12.2 67.0±26.9 RHC Systolic PAP (mm Hg), (SD) 49.7±15.1 56.2±15.9 0.15 Diastolic 16.6±3.3 19.6±8.2 mPAP 30.7±6.2 34.1±10 0.18 RAP 7.0±3.5 6.9±2.2 0.95 PVR WU 4.9±3.9 5.3±2.1 0.74 PCWP (SD 11.5±3.0 11.2±3.6 0.70 CO (L/min), 5.0±1.3 3.9±1.2 0.02 CI (L/min/m2), 2.7±0.6 2.2±0.8 0.13 Respiratory tests FVC (mL), 2241.7±460.7 1769.2±269.7 0.001 DLCO (%), 51.1±22.5 42±11 0.1 MWT, 270±160 200±164 0.23 initial significantly high, cardiac output (CO), values lower who died had more UIP patterns compared survived (Table 1). Initial PAH-specific therapy monotherapy 31 (70.5%) dual 11 (25%) Fifteen (34%) switched combined (dual or triple) monotherapy. Nine/fourteen (22.5%) on 13/26 (32.5%) died. Four could be specific various reasons. Bosentan (11.4%) monotherapy, ambrisentan tadalafil (9.1%) preferred follow-up. Median time better (44 vs. 61 months, p=0.01) (Figure Conclusion Our cohort consisted significant group, ILD especially pattern, anti-Scl-70 positivity, low baseline values. FVC, CO, 6-MWT affecting rates, poor long-term warranted need early effective modalities. REFERENCES: NIL. Acknowledgements: Disclosure Interests None Declared.