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

Judit Végh, G. Soós, I. Csípő, Nóra Demeter, Thomas Ben, B. Dezső, M. Zeher, Katalin Dévényi, J. Gaál, G. Szegedi, E. Bodolay

Research output: Contribution to journalArticle

16 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)264-269
Number of pages6
JournalRheumatology International
Volume26
Issue number3
DOIs
Publication statusPublished - Jan 2006

Fingerprint

Mixed Connective Tissue Disease
Iloprost
Pulmonary Hypertension
Lung
Doppler Echocardiography
Low Molecular Weight Heparin
Epoprostenol
Immunosuppressive Agents
Cardiac Catheterization
Heart Arrest
Pulmonary Embolism
Cyclophosphamide
Pulmonary Artery
Echocardiography
Arterial Pressure
Adrenal Cortex Hormones
Therapeutics
Biopsy
Pressure

Keywords

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

ASJC Scopus subject areas

  • Rheumatology

Cite this

Pulmonary arterial hypertension in mixed connective tissue disease : Successful treatment with Iloprost. / Végh, Judit; Soós, G.; Csípő, I.; Demeter, Nóra; Ben, Thomas; Dezső, B.; Zeher, M.; Dévényi, Katalin; Gaál, J.; Szegedi, G.; Bodolay, E.

In: Rheumatology International, Vol. 26, No. 3, 01.2006, p. 264-269.

Research output: Contribution to journalArticle

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