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THU0307 Pulmonary Arterial Hypertension & Systemic Lupus Erythematosus: Clinical Profile
  1. C. O. Ciang1,
  2. M. H. Leung1
  1. 1Department of Medicine, Queen Elizabeth Hospital, Hong Kong, Hong Kong


Objectives A retrospective case-control study to estimate prevalence of pulmonary arterial hypertension (PAH) in systemic lupus erythematosus (SLE) and to study the associated clinical variables.

Methods Clinical records of SLE patients followed up from 2006 to 2011 were retrieved. PAH was diagnosed by echocardiogram (systolic pulmonary artery pressure ≧ 40 mmHg)(1) or cardiac catheterization (mean pulmonary artery pressure ≧ 25 mmHg)(2). Controls were randomly selected from SLE patients with echocardiogram showing no PAH. PAH case: control ratio is 1:2 matching for age and gender. Clinical and serologic profiles were analyzed.

Results Thirty nine PAH patients were identified, the prevalence was 10.1%. The mean onset of PAH was 7.5 ± 8.3 (mean ± SD) years after SLE diagnosis. Compared with controls, significantly more PAH patientss had pericardial effusion (43.6 vs 15.9%), pleural effusion (43.6 vs 13%), psychosis (12.8 vs 1.4%), interstitial lung disease (25.6 vs 8.7%), hemolytic anemia (38.5 vs 14.5%), lower hemoglobin at presentation (10.5 ± 2.5 vs 11.8 ± 2.0 g/dL), higher anti- ds DNA at presentation (242.8 ± 204.1 vs 166.0 ± 121.6 IU/ml), proteinuria of more than 0.5g /day (76.9 vs 53.6%), worse serum creatinine at end of study (180.7 ± 236.6 vs 105.2 ± 98.8 umol/L), anti RNP positivity (57.9 vs 27.5%), higher SLEDAI (median of 4.0 vs 2.0) and SLICC(median of 2.0 vs 1.0). Significantly fewer PAH patients had malar rash (46.2 vs 79.7%) and photosensitivity (7.7 vs 39.1%). Clinical pattern of anti RNP positivity with negative malar rash gave odds ratio of 5.60 for PAH. These two variables remained significant after multiple logistic regression. PAH group had higher right ventricular systolic pressure (RVSP) than controls in echocardiogram, with mean RVSP of 51.7 ± 15.4 mmHg vs 25.3 ± 5.2 mmHg (p <0.001). In PAH group, deceased patients had higher RVSP than surviving patients (60.4 ± 8.9 mmHg vs 50.4 ± 15.9 mmHg with p = 0.034). There was no significant difference in RVSP among those who were symptomatic of PAH and those who were asymptomatic (p = 0.195)

Conclusions Clinical association of PAH with anti RNP positivity and absence of malar rash was noted. Awareness of factors associated with PAH in SLE aids early recognition and prompt treatment.


  1. Galiè N, Hoeper MM, Humbert M, et al. ESC Committee for Practice Guidelines. Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2009 Oct;30(20):2493-537

  2. Hoeper MM, Lee SH, Voswinckel R, et al. Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers. J Am Coll Cardiol 2006;48:2546–2552

Disclosure of Interest None Declared

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