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

Respiration. 2010;80(5):435-8. doi: 10.1159/000314587. Epub 2010 May 6.


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 after administration of low-dose epoprostenol in the intensive care unit. An autopsy revealed the patient suffered from pulmonary capillary hemangiomatosis. We review our case and what is known about this rare disease.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Antihypertensive Agents / administration & dosage
  • Antihypertensive Agents / adverse effects*
  • Autopsy
  • CREST Syndrome / complications*
  • Epoprostenol / administration & dosage
  • Epoprostenol / adverse effects*
  • Fatal Outcome
  • Female
  • Hemangioma, Capillary / diagnosis*
  • Humans
  • Hypertension, Pulmonary / etiology
  • Lung Diseases, Interstitial / complications
  • Lung Neoplasms / diagnosis*
  • Pulmonary Edema / chemically induced
  • Pulmonary Veno-Occlusive Disease / diagnosis


  • Antihypertensive Agents
  • Epoprostenol