Pulmonary hypertension in scleroderma and its relation to disease activity

Abstract

AimThe aim of this study was to screen for pulmonary hypertension (PH) in scleroderma patients using Doppler echocardiography, and correlate it with inflammatory parameters of the disease.Patients and methodsDoppler echocardiography was performed in 39 patients with scleroderma (three men and 36 women) and was compared with 20 age-matched and sex-matched healthy controls. Fifteen (38.5%) patients had limited scleroderma and 24 (61.5%) patients had diffuse scleroderma. PH was diagnosed if the peak systolic pressure gradient at the tricuspid valve was more than 25 mmHg. All patients were subjected to full assessment of history, rheumatological examination, laboratory studies, chest radiography, and pulmonary function tests.ResultsIn 39 scleroderma patients, PH was detected in five patients (12.8%); four of them had limited scleroderma and one had diffuse scleroderma. The range of pulmonary artery systolic pressure was 37–63 mmHg (mean ± SD = 49.2 ± 10.1). There was a highly statistically significant difference between patients and controls (P< 0.0001). There were no significant differences between patients with and without PH in clinical features, except for arthritis and Raynaud’s phenomena (P < 0.04, 0.01). Significant differences in the level of erythrocyte sedimentation rate, complement, and anticentromere antibodies were found in patients with PH versus those without PH (P < 0.04, <0.03, <0.002, respectively). There was a statistically significant correlation between pulmonary artery pressure with disease duration (P < 0.001) and Valentini Disease Activity Index (P< 0.001).ConclusionPatients with scleroderma have an increased risk of pulmonary arterial hypertension. Echocardiography should be used as a screening tool in patients who are at a higher risk of developing PH.