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THU0224 Pulmonary Arterial Hypertension in Patients with Juvenile Lupus Erythematosus
  1. A. Adrovic1,
  2. R. Dedeoglu2,
  3. S. Sahin1,
  4. K. Barut1,
  5. A. Koka2,
  6. O. Kasapcopur1
  1. 1Pediatric Rheumatology
  2. 2Pediatric Cardiology, Cerrahpasa Medical School, Istanbul, Turkey

Abstract

Background Juvenile systemic lupus erythematosus (jSLE) is a chronic autoimmune disease characterized with multisystemic involvement. Vital organ involvement is the most important morbidity and mortality factor. Early diagnosis of cardio-vascular and pulmonary involvement is shown to be relevant in term of reducing mortality.

Objectives The aim of this study is to explore the frequency of pulmonary hypertension in patients with jSLE, using non-invasive methods (Doppler echocardiography).

Methods Patients with diagnosis of jSLE followed up at our department were included in the study. Doppler echocardiography was performed at all included patients. Pulmonary arterial pressure was determineted by three different methods. Systolic pulmonary arterial pressure (SPAP) was estimated by measurement the maximal velocity of tricuspid insufficiency (TI). Diastolic pulmonary arterial pressure (DPAP) was estimated by velocity of pulmonary insufficiency at the end of diastole (PI). Mean pulmonary arterial pressure (MPAP) was determinated by pulmonary insufficiency acceleration time (AT) and the ration of AT to ejection time (ET). Systolic pulmonary arterial pressure was calculated by modified Bernoulli equation. Right atrial pressure was evaluated by measurement the right atrial volume, the degree of tricuspid regurgitation and contraction of vena cava inferior during inspiration

Results A total of 38 jSLE patients were included in the study. Mean age of patient was 16±2,59 year, mean age at diagnosis was 10,6±3,5 year and mean disease duration was 57±33,6 month. Velocity of pulmonary insufficiency at the end of diastole (PI) was 1,469±0,295 m/sec and 1,214±0,128 m/sec in patients and control group, respectively. Maximal velocity of tricuspid insufficiency (TI) was 2,340±0,277 m/sec and 2,044±0,411 m/sec in patients and control group, respectively.The ration of pulmonary insufficiency acceleration time (AT) to ejection time (ET) was normal in both patients and in control group. Although estimated pulmonary arterial pressure was found to be in normal ranges (<25 mmHg) in all studies patients, it was statistical significantly higher comparing to control group. According to those results, there were no findings of pulmonary hypertension among patients with jSLE.

Conclusions This study confirms that pulmonary hypertension is uncommon among patients with jSLE. Early diagnosis, regular follow up and proper therapy are thought to be important in reducing the risk of pulmonary hypertension.

  1. Kasparian A, Floros A, Gialafos E, Kanakis M, Tassiopoulos S, Kafasi N, et al. Raynaud's phenomenon is correlated with elevated systolic pulmonary arterial pressure in patients with systemic lupus erythematosus. Lupus 2007;16:505–8.

  2. Prabu A, Gordon C. Pulmonary arterial hypertension in SLE: what do we know? Lupus 2013;22:1274–85.

  3. Min HK, Lee JH, Jung SM, Lee J, Kang KY, Kwok SK, et al. Pulmonary hypertension in systemic lupus erythematosus: an independent predictor of patient survival. Korean J Intern Med 2015;30:232–41.

  4. Kamel SR, Omar GM, Darwish AF, Asklany HT, Ellabban AS, et al. Asymptomatic pulmonary hypertension in systemic lupus erythematosus. Clin Med Insights Arthritis Musculoskelet Disord. 2011;4:77–86.

Disclosure of Interest None declared

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