AB0448 SYSTEMIC SCLEROSIS ASSOCIATED PULMONARY ARTERIAL HYPERTENSION: PREDOMINANCE OF PULMONARY FIBROSIS AS A RISK FACTOR FOR MORTALITY IN A SINGLE CENTER COHORT
Authors: S. Amikishiyev, Y. Yalçinkaya, N. Aliyeva, K. Mammadova, B. Artim-Esen, A. Gül, A. K. Bilge, G. Okumuş, M. Inanc
Journal: Annals of the Rheumatic Diseases (2021)

Background: Pulmonary arterial hypertension (PAH) is a severe vasculopathic complication for systemic sclerosis (SSc) patients. The availability of oral-specific vasodilator therapies has provided better outcomes. Objectives: We aimed to analyze the characteristics of SSc-PAH patients and factors associated with mortality. Methods: Medical records of 291 SSc patients fulfilling ACR/EULAR criteria (2013) and followed–up during 2008-2020 years were screened and the patients who diagnosed PAH evaluated by right heart catheterization (mean PAB≥20 mmHg) (n=26, 8,9%) were included into this retrospective cross-sectional study. Results: The characteristics of 26 SSc-PAH(24 females) patients were summarised in Table 1. Table 1. Characteristics of SSc patients with PAH SSc-PAH (n=26 ) Demographics Age(yrs) 53.6±8.9 Duration of Raynaud’s(yrs) 16.1±11.8 Duration of Non-Raynaud’s(yrs) 9.2±6.6 Raynaud’s to Diagnosis of PAH (yrs) 10.8±6.7 Clinical Characteristics (% ) LcSSc 9 (34.6) DcSSc 18 (65.4) Digital ulcer 14 (53.8) Gastrointestinal 20 (69.0) Synovitis 5(19.2) Flex contractures 7 (26.9) Tendon friction rubs 3(11.5) Renal crisis 1 (3.8) Pulmonary fibrosis 19 (73.1) Auto-antibodies (% ) ANA 23 (88.5) Anti-centromere 6(23.1) Anti-Scl70 13(50) Treatment (% ) Specific vasodilator ERA (bosentan/macicentan/ambricentan) 15 (51.7) PDE5-i (sildenafil/tadalafil) 17 (58.6) Prostacyclin-analog (İloprost/treprostenil/selexipag) 15 (51.7) Riociguat 5 (17.2) Immunosuppressives 21(80.8) Steroids 16(61.5) Twenty-four (92,3%) of the SSc-PAH patients had PAH-related symptoms at the time of diagnosis, 2 (7,7%) were asymptomatic and diagnosed by screening. RHC and treatment details were stated in Table 2. Table 2. Right Heart Catheterization (RHC) ve treatment details of SSc-PAH patients n=26 Initial RHC -mean PAB 30,4±7,9 (median 28, 20-53mmHg) -PVR 5,1±2,4 (median 4, 3-9 woods) -PCWP 10,8±5,5 (median 10, 0-15 mmHg) Initial treatment -monotherapy 5 (19,2%) -combination 10 (38,5%) -add-on combination 12 (46,2%) Eleven out of 26 patients (42,3 %) were deceased after a mean follow up of 43,7±24,6 (median 48,1-84) from PAH diagnosis and 15,1±9,9 (median 13, 0,6-34) years after SSc diagnosis. Deceased patients were younger and had younger age at disease onset (49,1± 8,8 vs 56,9±7,7, p=0.032 and 30,8±13,0 vs 42.0 ±10,9, 0.027). All deceased patients had associated pulmonary fibrosis (100 vs 53.3%, p=0.01). No significant difference was observed for initial RHC parameters between deceased and survived SSc-PAH patients. Specific monotherapy was found to be more frequent in deceased patients (45,5 vs 0%, p=0,007). Conclusion: The prevalence of SSc-PAH was found to be 8.9% with increased mortality in our cohort. SSc-PAH patients predominantly had diffuse cutaneous involvement with digital vasculopathy, pulmonary fibrosis, and anti-Scl70 positivity. PAH was diagnosed after a median of 10 years of the Raynaud’s, mainly in symptomatic patients. Mortality in PAH-SSc patients was associated with early onset of disease, pulmonary fibrosis, and monotherapy. Initial RHC parameters were not found to be related to mortality. Disclosure of Interests: None declared.