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

Franklin R. McGuire | Zendy; Tina Kennelly | Zendy; Thomas W. Tillack | Zendy; Mark Robbins | Zendy

AI Assistant Blog Pricing

Home ZAIA Blog

Open Access

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

Author(s) -

Franklin R. McGuire,

Tina Kennelly,

Thomas W. Tillack,

Mark Robbins

Publication year - 2010

Publication title -

respiration

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 1.264

H-Index - 81

eISSN - 1423-0356

pISSN - 0025-7931

DOI - 10.1159/000314587

Subject(s) - medicine , pulmonary edema , rare disease , pulmonary hypertension , autopsy , respiratory disease , lung , disease , surgery , cardiology

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.

The content you want is available to Zendy users.

Already have an account? Click here to sign in.

Having issues? You can contact us here

Accelerating Research