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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
Characteristics of SSc patients with PAH
|
SSc-PAH
|
|
| 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
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.