Pulmonary Capillary Hemangiomatosis Associated with CREST Syndrome: A Case Report and Review of the Literature

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This is a report of fatality immediately after administration of epoprostenol. The patient was previously diagnosed with CREST syndrome and associated interstitial lung disease. She developed worsening pulmonary hypertension and was clinically diagnosed with pulmonary veno-occlusive disease. The patient developed flash pulmonary edema and arrested Received: June 25, 2009 Accepted after revision: January 4, 2010 Published online: May 6, 2010 Franklin McGuire, MD University of South Carolina School of Medicine 8 Medical Park, Suite 410 Columbia, SC 29203 (USA) Tel. +1 803 799 5022, Fax +1 803 799 5890, E-Mail franklin.mcguire @ uscmed.sc.edu © 2010 S. Karger AG, Basel 0025–7931/10/0805–0435$26.00/0 Accessible online at: www.karger.com/res Established Facts • Both CREST disease and pulmonary capillary hemangiomatosis (PCH) are uncommon disease states, but both clinical entities occurring together are a rarity. • Patients with pulmonary hypertension may be treated with long-term epoprostenol to improve their symptoms.

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Pulmonary capillary hemangiomatosis associated with CREST syndrome: a case report and review of the literature.

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تاریخ انتشار 2010