Pulmonary arterial hypertension in systemic sclerosis (PAH-SSc) in comparison with idiopathic PAH (IPAH) is characterized by rapid progression, higher mortality and poor response to PAH-specific therapy.
51 patients with PAH-SSc and 50 patients with IPAH. Univariate logisitic regression was used to calculate the probability (odds ratio (OR)) of SSc symptoms.
We identified 29 symptoms that were associated with PAH-SSc. The most significant of them, increasing the risk of detection of SSc in patients with PAH, were age>45 years (OR 9.7, 95% CI 3.9-24.3, p<0.001), diffusion lung capacity (DLCO)<60% (OR 13.8, 95% CI 4.3-44.7, p<0.0001), ratio of forced vital capacity to DLCO<1.7 (OR 13.0, 95% CI 3.9-42.9, p <0.00001), C-reactive protein>2 mg/l (OR 12.9, 95% CI 3.96-41.8, p<0.001), uric acid level>387 μmol/L (OR 3.8, 95% CI 1.5-9.6, p=0.003), pericardial effusion (OR 2.3, 95% CI 1.0-5.1, p=0.04), mean right ventricular pressure>15.5 mmHg (OR 8.9, 95% CI 3.4-23.1, p<0.001), diastolic right ventricular pressure>4.5 mmHg (OR 4.6, 95% CI 1.9-11.2, p=0.003). Symptoms that reduce the risk of detecting SSc include hemoglobin level >146 g/l (OR 0.3, 95% CI 0.2-0.8, p=0.007), syncope (OR 0.3, 95% CI 0.1-0.9, p=0.02), mean pulmonary artery pressure>55 mmHg (OR 0.4, 95% CI 0.2-0.9, p=0.03), pulmonary vascular resistance>12 units Wood (OR 0.4, 95% CI 0.2-0.9, p=0.02).
The identification of specific signs of PAH will allow early diagnosis of SSc, predict the course of PAH, and more carefully approach the choice of PAH therapy. What will extend the life of patients with PAH-SSc.