Massive intractable hemoptysis due to idiopathic granulomatous pulmonary veno-occlusive disease.
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.
Department of Medicine, University of Washington School of Medicine, Seattle, Washington 98104, USA. tewest@uw.edu
T Eoin
TE
West
C Dirk
CD
Keene
Rodney A
RA
Schmidt
Kelly D
KD
Smith
D000328:Adult; D017809:Fatal Outcome; D006099:Granuloma / Q000150:complications*; D006469:Hemoptysis / Q000209:etiology*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology; D007249:Inflammation / Q000150:complications; D008297:Male; D011668:Pulmonary Veno-Occlusive Disease / Q000150:complications* / Q000473:pathology*; D012128:Respiratory Distress Syndrome / Q000209:etiology; D014699:Venules / Q000473:pathology*