Objectives: To describe the frequency of patients with an elevated systolic pulmonary artery pressure (sPAP) estimated by Doppler echocardiography in a population of SLE patients followed in a tertiary reference centre.
Methods: A search of our Internal Medicine Department database identified 93 SLE patients followed between 1995 and 2005. Their medical records were reviewed retrospectively. The PH threshold was defined as sPAP≥35mmHg. Characteristics of PH and non-PH SLE patients were compared using Fisher's, chi-square or Wilcoxon's exact test.
Results: Elevated sPAP was detected in 12/93 (13%) patients. When analysing the mechanisms of PH, it was considered as secondary to specific lung involvement in 2 cases, due to severe left ventricular dysfunction in 1 patient and probably corresponding to SLE-associated PAH in the 9 remaining subjects. Univariate analyses showed that sPAP≥35mmHg was more common in Black subjects (50 vs. 20%, p=0.03), in patients with longer disease duration (14±8 vs. 9.5±8 years, p=0.049), and in patients with a history of peripheral nervous system involvement (25 vs. 4%, p=0.02), pericarditis (58 vs. 27%, p=0.04), anti-Sm (42 vs. 11%, p=0.01), and anticardiolipin antibodies (75 vs. 31% p=0.007).
Conclusions: PH is a relatively common complication of SLE patients managed in tertiary care centres. Doppler echocardiography allows non-invasive detection of elevated sPAP in this population that should then benefit from gold-standard techniques including right-heart catheterisation in order to confirm the diagnosis, as well as the cause and severity of PH.