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Diagnosis of pulmonary arterial hypertension in a patient with systemic sclerosis

Nature Clinical Practice Rheumatology volume 4, pages 160–164 (2008)Cite this article

Abstract

Background A 42-year-old woman with limited cutaneous systemic sclerosis presented with rapid-onset dyspnea on exertion, which had developed over the previous 8 weeks. She had not experienced any dyspnea before this period. Transthoracic Doppler echocardiography performed 6 months before presentation demonstrated an estimated right ventricular systolic pressure of 32 mmHg. Lung function tests also performed at that time revealed a decreased diffusion capacity for carbon monoxide of 54% and normal lung volumes, and high-resolution CT scan of the lungs was normal.

Investigations Physical investigation, CBC, analysis of C-reactive protein and pro-brain natriuretic peptide, transthoracic Doppler echocardiography, six-minute walk test, lung function tests including diffusion capacity for carbon monoxide, right heart catheter, high-resolution CT scan, and ventilation/perfusion scan.

Diagnosis Pulmonary arterial hypertension associated with limited cutaneous systemic sclerosis.

Management Treatment with oral anticoagulation therapy and the endothelin-receptor antagonist bosentan. Monitoring of adverse effects of bosentan therapy was performed using liver function tests.

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Figure 1: Chest X-rays of a patient with systemic sclerosis and end-stage pulmonary arterial hypertension.

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Author information

Authors and Affiliations

  1. JHW Distler is a Fellow and Research Group Leader at the Department of Internal Medicine 3, University of Erlangen-Nuremberg, Hanover, Germany,

    Jörg HW Distler

  2. MM Hoeper is Associate Professor at the Department of Respiratory Medicine, Hanover Medical School, Hanover, Germany,

    Marius M Hoeper

  3. O Distler is a Lecturer and Consultant at the Department of Rheumatology and Center of Experimental Rheumatology/Integrative Human Physiology, University Hospital Zurich, Switzerland.,

    Oliver Distler

Authors
  1. Jörg HW Distler
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  2. Marius M Hoeper
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  3. Oliver Distler
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Corresponding author

Correspondence to Oliver Distler.

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Competing interests

JHW Distler has received speaking fees and is a consultant for Encysive Pharmaceuticals and Actelion Pharmaceuticals. MM Hoeper has received speaking fees, grant/research support and is a consultant for Encysive Pharmaceuticals, Actelion Pharmaceuticals, GlaxoSmithKline, Pfizer and Bayer-Schering Pharma. O Distler has received speaking fees and is a consultant for Encysive Pharmaceuticals, Actelion Pharmaceuticals, Array Biopharma and Ergonex Pharma.

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Distler, J., Hoeper, M. & Distler, O. Diagnosis of pulmonary arterial hypertension in a patient with systemic sclerosis. Nat Rev Rheumatol 4, 160–164 (2008). https://doi.org/10.1038/ncprheum0728

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