Rheumatology Advance Access published online on August 11, 2009
Rheumatology, doi:10.1093/rheumatology/kep203
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Prevalence and risk factors for pulmonary arterial hypertension in patients with lupus
1Department of Rheumatology, Division of Immunity and Infection, University of Birmingham, 2Department of Rheumatology, University of Birmingham NHS Foundation Trust, 3Department of Rheumatology, Sandwell and West Birmingham NHS Trust, Birmingham, 4Department of Rheumatology, Worcestershire Acute Hospitals NHS Trust, Worcester, 5Department of Cardiology, Sandwell and West Birmingham NHS Trust, 6Department of Cardiology, University of Birmingham, 7Wellcome Trust Clinical Research Facility, Queen Elizabeth Hospital and 8Lung Injury and Fibrosis Treatment Programme, Department of Medical Sciences, University of Birmingham, Birmingham, UK.
Correspondence to:
Caroline Gordon, Department of Rheumatology, Division of Infection and Immunology, The Medical School (East Wing), University of Birmingham, Birmingham B15 2TT, UK. E-mail: p.c.gordon{at}bham.ac.uk
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Abstract |
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Objectives. Pulmonary arterial hypertension (PAH) is associated with rapid deterioration and poor prognosis in SLE, especially during pregnancy. The prevalence of PAH in SLE in non-tertiary centres is uncertain. This study aims to estimate the point prevalence of PAH and identify risk factors for PAH in a large cohort of SLE patients.
Methods. A prospective cross-sectional study of 288 patients with SLE were recruited from lupus clinics in Birmingham, UK. Resting transthoracic echocardiography was performed to estimate the pulmonary artery pressures and to assess cardiac morphology and function. PAH was defined as systolic pulmonary artery pressure (sPAP) >30 mmHg. We assessed potential risk factors such as the presence of lung disease, respiratory muscle weakness, autoantibodies, smoking, RP and APS.
Results. Of 288 patients who consented for participation, 283 patients were suitable for analysis. Twelve patients were found to have PAH with sPAP >30 mmHg. The range of sPAP in our PAH patients was 31–59 mmHg and three patients had sPAP >40 mmHg. The only significant risk factor for PAH was LAC (P = 0.005).
Conclusions. The point prevalence of PAH was 4.2% in our cohort of patients with SLE. Most of the PAH cases were found to be of mild severity (<40 mmHg). The significant association of LAC and presence of APS in PAH cases suggests that thrombosis may play an important role in PAH with SLE. This is important, as it is treatable.
KEY WORDS: Systemic lupus erythematosus, Pulmonary arterial hypertension, Prevalence, Echocardiography, Lupus anti coagulant, Screening, Risk factors, Anti-phospholipid antibodies
Submitted 15 December 2008;
revised version accepted 12 June 2009.
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