Massive intractable hemoptysis due to idiopathic granulomatous pulmonary veno-occlusive disease

Respir Care. 2013 May;58(5):e56-9. doi: 10.4187/respcare.02084.

Abstract

Pulmonary veno-occlusive disease is a rare condition with limited treatment options. The pathological hallmark of the disease is occlusion of pulmonary venules and small veins in the lobular septa. The etiology of the disease remains obscure. We report and discuss an extremely unusual case presenting as massive and intractable hemoptysis, in which pulmonary venous occlusion was attributed to granulomatous venulitis in the absence of other pulmonary or systemic inflammatory abnormalities.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Fatal Outcome
  • Granuloma / complications*
  • Hemoptysis / etiology*
  • Humans
  • Hypertension, Pulmonary / etiology
  • Inflammation / complications
  • Male
  • Pulmonary Veno-Occlusive Disease / complications*
  • Pulmonary Veno-Occlusive Disease / pathology*
  • Respiratory Distress Syndrome / etiology
  • Venules / pathology*