Author + information
- Uğur Nadir Karakulak1,
- Naresh Maharjan1,
- Elifcan Aladağ2,
- Levent Kılıç2,
- Ali Akdoğan2,
- Ergün Barış Kaya1 and
- Lale Tokgözoğlu1
Despite improved treatment modalities, the prognosis of pulmonary hypertension due to connective tissue disease such as scleroderma is very high. In this study, we evaluate factors that affect pulmonary artery pressure in scleroderma patients with pulmonary hypertension
24 scleroderma patients with pulmonary hypertension were included in this study. Detailed history was taken, physical examination and electrocardiography performed and pulmonary hypertension was diagnosed by transthoracic echocardiography along with right heart catheterization during which systolic, diastolic and mean pulmonary artery pressures were measured. High resolution tomography was used to diagnose interstitial pulmonary disease and inflammatory markers and autoantibodies were also measured. Factors that can be affected on pulmonary artery pressure were evaluated with Spearman's rho test and Cox regression analysis.
A total of 24 patients, 19 were women (79%). In univariate analysis, there is a positive correlation between pulmoary artery pressure and body mass index, right ventricular diastolic diameter and BNP levels and also there is a negative correlation between pulmonary artery pressure and presence of hypertension and white blood cell count. In multivariate analysis, there is positive correlation between only right ventricular diastolic diameter (r=0,649, p<0,0001) and negative correlation between presence of hypertension (r=-0,502, p=0,002).
Pulmonary hypertension accompanying scleroderma seriously decreases the survival of these patients. Pulmoary artery pressure is an important factor in these patients’ clinical course. Thus patients who have more elevated pulmonary artery pressure may be follewed earlier, more intense and with combination therapy. In this study, it was shown that there are strong correlations between right ventricular diastolic diameter, presence of hypertension and pulmonary artery pressure in scleroderma patients with pulmonary hypertension.