Pulmonary hypertension in systemic lupus erythematosus

Lee, Hwa Jung; Kim, Hyeon Jeong; Lee, Choong Won

Abstract

BACKGROUND: Pulmonary arterial hypertension (PAH) in systemic lupus erythematosus (SLE) has very poor prognosis and the prevalence of the disease is also not well delineated. This study was designed to assess the prevalence of PAH in asymptomatic SLE patients and to analyze the potential association between the presence of PAH and SLE including disease activity, organ involvement, and serology.
METHODS
Asymptomatic 50 SLE patients and 50 healthy controls of a similar age and sex were assessed by an echocardiographic and clinical study. The diagnosis of PAH was made when the right ventricular systolic pressure (RVSP), measured by 2-dimensional echocardiography, was > or =40 mmHg.
RESULTS
Compared to control subjects, lupus patients had significantly increased RVSP (mean+/-SD, 34.9+/-8.9 vs 31.2+/-3.6 mmHg, p=0.008). Patients with SLE were divided into three groups: RVSP> or =40 mmHg, RVSP=30~39 mmHg, RVSP<30 mmHg. Seven patients (14%) had RVSP> or =40 mmHg, 36 patients (72%) had RVSP=30~39 mmHg and 7 patients (14%) had RVSP<30 mmHg. In the healthy controls, none of them had RVSP> or =40 mm Hg. Of the controls, 43 (86%) had RVSP=30~39 mmHg and 7 (14%) had RVSP<30 mmHg. There was no statistical difference in organ involvement, Raynaud's phenomenon, vasculitis, anti-RNP and anti-cardiolipin Ab among the three SLE groups. However, lupus disease activity (checked by SLEDAI-2K) was associated with PAH (p=0.014).
CONCLUSIONS
The prevalence of PAH as seen on echocardiograms in SLE patients was 14%. Organ involvement, Raynaud's phenomenon, vasculitis, antiphopholipid antibodies and anti-RNP except SLEDAI-2K were not associated with pulmonary arterial pressure.