Pulmonary arterial hypertension in mixed connective tissue disease: successful treatment with Iloprost
| Autor: | Balazs Dezso, Katalin Dévényi, Gyula Szegedi, Margit Zeher, Thomas Ben, Istvan Csipo, Edit Bodolay, Nora Demeter, Györgyike Soós, János Gaál, Judit Végh |
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| Rok vydání: | 2005 |
| Předmět: |
medicine.medical_specialty
Biopsy Hypertension Pulmonary Vasodilator Agents Immunology Doppler echocardiography Mixed connective tissue disease Rheumatology medicine.artery Internal medicine medicine Humans Immunology and Allergy Iloprost Autoantibodies Mixed Connective Tissue Disease medicine.diagnostic_test business.industry Respiratory disease Middle Aged medicine.disease Pulmonary hypertension Echocardiography Doppler Pulmonary embolism Surgery medicine.anatomical_structure Pulmonary artery Cardiology Female business Artery medicine.drug |
| Zdroj: | Rheumatology International. 26:264-269 |
| ISSN: | 1437-160X 0172-8172 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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