Abstract
Background: Pulmonary arterial hypertension has been reported between 0.5 and 14% in systemic lupus erythematosus (SLE).
Objectives: To assess PH prevalence, etiology and risk factors in a SLE cohort.
Methods: Prospective cross-sectional study of 158 SLE patients. Doppler echocardiographic (DE), diffusing capacity for CO (DLCO), NtproBNP and dyspnea (Borg scale) were performed in all patients. An echocardiographic exercise test (EE) was conducted in selected patients. When sPAP ≥ 45 mmHg (DE) or a positive EE (>20 mmHg increase in PAPs) a right heart catheterization (RHC) at rest or during exercise was performed. A rest mean pulmonary pressure (mPP) ≥25 mmHg was accepted as PH. When rest mPP was less than 25 mmHg, an exercise test was conducted. Patients with resting PH (sPAP ≥ 35 and < 45 mmHg) and obvious cardiac disease were excluded from RHC.
Results: Mean age: 45±12.9 years, 94.3% females. Twenty one patients (13.4%) had dyspnea (Borg scale ≥ 2). Eleven patients (6.9%) showed any degree of PH. Eight patients (out of 11) had PH of left cardiac origin. One patient had thromboembolic disease. Two patients had precapillary PH related with SLE. All 11 patients with PH had dyspnea (Borg scale ≥ 2) vs. those without PH (p<0.001). PH patients showed a significant decrease in DLCO and higher NtproBNP. There were no differences in SLE clinical characteristics between SLE patients and those without PH.
Conclusions: Our data confirm the low prevalence of precapillary PH in SLE. We found a preponderance of cardiac etiology. A PH screening program based on DE, NtproBNP and DLCO not seems to be cost-effective and should be restricted to SLE patients with unexplained dyspnea.
- © 2012 ERS
