Author + information
- Uğur Nadir Karakulak1,
- Naresh Maharjan1,
- Elifcan Aladağ2,
- Levent Kılıç2,
- Ali Akdoğan2,
- Ergün Barış Kaya1 and
- Lale Tokgözoğlu1
Scleroderma is a chronic inflammatory and autoimmune disease that leads to fibrosis of the skin and visceral organs. Although interstitial pulmonary fibrosis is the most common pulmonary manifestation, pulmonary hypertension due to pulmonary vasculopathy is also seen frequently. It is important to determine patients at risk of developing pulmonary hypertension for prognostic reasons. In this study, risk factors and predictors associated with the development of pulmonary hypertension in scleroderma patients were studied.
A total of 160 patents were included in this study. A detail history was taken, physical examination was performed, electrocardiography was obtained and pulmonary hypertension was diagnosed first by transthoracic echocardiography which was then followed by right heart catheterization where the systolic, diastolic and mean pulmonary artery pressures were obtained. High resolution computed tomography was used to scan for interstitial pulmonary disease; besides routine biochemistry, inflammatory parameters and autoantibodies were also determined.
Pulmonary hypertension was found in 15% of the patients. In univariate analysis, the degree of tricuspid regurgitation, right ventricular diastolic diameter, presence of pericardial effusion, pulmonary artery diameter in tomography, hemoglobin and BNP levels were found to be associated with the development of pulmonary hypertension. In multivariate analysis, only pulmonary artery diameter measured in tomography and the degree of tricuspid regurgitation measured by echocardiography were found to independent predictors of pulmonary hypertension (p values of 0.002 and <0.001 respectively). The risk of development of pulmonary hypertension was higher in patients with a pulmonary artery diameter >27 mm as compared to those with a diameter ≤27 mm (Fig. 1 and 2). The sensitivity of pulmonary artery diameter as a predictor was 79.1% and specificity was 92.6%, positive predictive value was 65.5% and negative predictive value was 96.2%.
Pulmonary hypertension accompanying scleroderma causes a significant decrease in survival of these patients. The determination of patients at risk of developing pulmonary hypertension is very important for intensification of therapy and early intervention. In this study, pulmonary artery diameter measured in computed tomography was found to predict scleroderma patients at risk of developing pulmonary hypertension with a high accuracy and early in the course of the disease.