Long-Term Follow-Up Of Patients With Systemic Sclerosis And Normal Or Borderline Pulmonary Pressures

Accumulating evidence from PAH associated with systemic sclerosis is teaching us that patients with PAMP between 21 and 24 mmHg have an increased risk of progression and have a higher mortality. We have analyzed retrospectively a cohort of 27 patients (25 females and 2 males) with systemic sclerosis who were catheterized for suspected pulmonary hypertension and their PAMP was 16-24. Significant interstitial lung involvement, left heart disease and chronic thrombotic obstruction of pulmonary vessels were excluded. Characteristics of patiensts at baseline were: mean age mean pulmonary artery pressure, mean pulmonary vascular resistance, mean FEV1, mean FVC, mean DLCO, mean proBNP. During the follow-up we evaluated the incidence of progression to pulmonary hypertension according to the current definition (PAMP ≥25 mmHg) and survival. During the median follow-up of 31.5 months, 4 (15 %) patients had progression to pulmonary hypertension as defined by current guidelines and 7 (26 %) patients died. The group of patients with PH-free survival differed statitically significantly in pulmonary vascular resistence (2.03±0.63 vs. 2.96±0.80, p=0.002) and NT-proBNP levels at baseline (278±252.1 vs. 597.5±351.5, p=0.03). Conclusion: Patients with SSc and normal or borderline pulmonary pressures ( PAMP 16-24 mmHg) are at high risk of progression and/or death. Patients with progression or death had statistically significantly higher pulmonary vascular resistence and NT-pro BNP levels at baseline.