Evaluation of pulmonary artery pressure in patients with juvenile systemic lupus erythematosus (jSLE).

Amra Adrovic,Dicle Cengiz,Funda Oztunc,Sezgin Sahin,Reyhan Dedeoglu,Ozgur Kasapcopur,Kenan Barut,Aida Koka

doi:10.17305/bjbms.2017.2178

Amra Adrovic, Dicle Cengiz + Show 6 more

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https://doi.org/10.17305/bjbms.2017.2178

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Abstract

Juvenile systemic lupus erythematosus (jSLE) is a chronic multisystemic autoimmune disease. Previous studies among adults have shown impaired right ventricular (RV) function in patients with SLE. Also, these patients may develop pulmonary artery hypertension (PAH), which is one of the most threatening complications of SLE. Nevertheless, studies on PAH among jSLE patients are still rare. The aim of this study was to assess the RV function in jSLE patients by Doppler echocardiography (Echo Doppler). We also estimated pulmonary artery systolic pressure (PASP) and mean pulmonary artery pressure (mPAP) in these patients. A total of 38 jSLE patients and 40 sex- and age-matched controls were retrospectively analyzed. All patients underwent combined M-mode, cross-sectional echo, and Doppler Echo examination. The RV function was significantly impaired in jSLE patients compared to controls. PASP and mPAP were normal in 37 out of 38 patients (97.37%), however, the mean values of PASP and mPAP were significantly higher in jSLE patients compared to controls (26.90 mmHg versus 21.71 mmHg and 12.63 mmHg versus 9.89 mmHg, respectively) [p < 0.05]. Only one patient (2.6 %) had elevated mPAP (60 mmHg). The right cardiac catheterization confirmed PAH in this patient. Although PAH was detected only in one patient, there was a marked increase of PAP in our jSLE patients. Overall, PASP and mPAP were significantly higher in jSLE patients compared to healthy controls. Prospective studies with ethnically diverse cohorts could give more insight on the relevance of PAP and PHT in patients with jSLE.

Highlights

Juvenile systemic lupus erythematosus is a chronic multisystemic autoimmune disease
pulmonary hypertension (PHT) is a progressive disease characterized by an increased mean pulmonary artery pressure
A total of 38 Juvenile systemic lupus erythematosus (jSLE) patients and 40 sex- and age-matched healthy controls were included in the study

Summary

Introduction

Juvenile systemic lupus erythematosus (jSLE) is a chronic multisystemic autoimmune disease. Vital organ involvement is among the most important risk factors of morbidity and mortality in jSLE, and early diagnosis of cardiovascular and pulmonary involvement has shown to reduce the mortality rate [1,2,3]. PHT is a progressive disease characterized by an increased mean pulmonary artery pressure (mPAP; >25 mmHg at rest or >30 mmHg. Submitted: 18 May 2017/Accepted: 22 June 2017 during exercise) [2], as well as by an elevated pulmonary artery systolic pressure (PASP; >30 mmHg at rest), estimated by Doppler echocardiography (Echo Doppler). In adult patients with SLE, a PASP >30 mmHg was indicative of PHT [2,3,4]

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