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Pulmonary arterial hypertension in mixed connective tissue disease: successful treatment with Iloprost

Rheumatology International volume 26, pages 264–269 (2006)Cite this article

Abstract

This paper describes a 61-year-old woman who presented with mixed connective tissue disease, which was complicated by the development of pulmonary arterial hypertension (PAH). Her condition worsened rapidly, with development of haemopthysis, tachypnoe and cardiac arrest. Doppler echocardiography showed a high systolic pulmonary arterial pressure (98 mmHg), confirmed by the right heart catheterization. Vasculopathy of the pulmonary artery vessels was detected following open lung biopsy. No pulmonary embolism was found. Because of suspicion of flare of her underlying disease, which leads to PAH, immunosuppressive treatment was started with high doses of corticosteroid and cyclophosphamide, in combination with the prostacyclin analogue, Iloprost, and low molecular weight heparin. The therapy resulted in slow recovery over 6 weeks, with control echocardiography showing normalization of the high pulmonary pressure, and the patient being capable of returning to everyday activities.

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Abbreviations

PAP:

Pulmonary arterial pressure

RA:

Right atrial

TR:

Tricuspid regurgitation

vWFAg (<120%):

von Willebrand Factor antigen

AECA (<35 U/ml):

Anti-endothelial cell antibodies

aU1RNP (<5 U/ml):

Anti-U1RNP antibody

TM (<3.9 ng/ml):

Thrombomodulin

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Affiliations

  1. Divisions of Clinical Immunology, 3rd Department of Internal Medicine, University of Debrecen Medical and Health Science Center, 22 Móricz Street, Debrecen, 4004, Hungary

    Judit Végh, István Csipõ, Nóra Demeter, Margit Zeher, Gyula Szegedi & Edit Bodolay

  2. Department of Pathology, University of Debrecen Medical and Health Science Center, 22 Móricz Street, Debrecen, 4004, Hungary

    Györgyike Soós & Balázs Dezsõ

  3. 1st Department of Internal Medicine, University of Debrecen Medical and Health Science Center, 22 Móricz Street, Debrecen, 4004, Hungary

    Thomas Ben

  4. Department of Radiology, University of Debrecen, Medical and Health Science Center, Debrecen, 4004, Hungary

    Katalin Dévényi

  5. Division of Rheumatology, Hospital Kenézy Gyula, Debrecen, 4004, Hungary

    János Gaál

Authors
  1. Judit Végh
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  2. Györgyike Soós
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  4. Nóra Demeter
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  5. Thomas Ben
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  6. Balázs Dezsõ
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  7. Margit Zeher
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  8. Katalin Dévényi
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  9. János Gaál
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  10. Gyula Szegedi
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  11. Edit Bodolay
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Correspondence to Edit Bodolay.

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Végh, J., Soós, G., Csipõ, I. et al. Pulmonary arterial hypertension in mixed connective tissue disease: successful treatment with Iloprost. Rheumatol Int 26, 264–269 (2006). https://doi.org/10.1007/s00296-005-0616-8

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  • DOI: https://doi.org/10.1007/s00296-005-0616-8

Keywords

  • Mixed connective tissue disease
  • Pulmonary arterial hypertension
  • Prostacyclin
  • Echocardiography