Pulmonary veno-occlusive disease

Palmer SM, Robinson LJ, Wang A, Gossage JR, Bashore T, Tapson VF. Massive pulmonary edema and death after prostacyclin infusion in a patient with pulmonary veno-occlusive disease. Chest 1998 l 13(1 ) 237 10. 

Pulmonary veno-occlusive disease and/or pulmonary capillary hemangiomatosis... 

PPH), heritable pulmonary hypertension, drug- and toxin-induced pulmonary hypertension, associated with known disease (APAH), persistent pulmonary hypertension of the newborn, and pulmonary veno-occlusive disease (PVOD) and/or pulmonary capillary hemangiomatosis (PCH). 

Other rare vascular complications of sarcoidosis (limited to a few case reports) include pulmonary arterial stenoses from granulomatous involvement of the vessels, extrinsic compression of pulmonary arteries by enlarged hilar lymph nodes or fibrosing mediastinitis (6), pulmonary veno-occlusive disease (resulting from obstruction of interlobular septa veins by granulomata or perivascular... 

Acute pulmonary edema Diffuse alveolar hemorrhage Periengraftment respiratory distress syndrome Bronchiolitis obliterans syndrome Bronchiolitis obliterans organizing pneumonia Idiopathic pulmonary syndrome Delayed pulmonary toxicity syndrome Pulmonary cytolytic thrombi Pulmonary veno-occlusive disease Progressive pulmonary fibrosis Pulmonary hypertension Hepatopuhnonary S3mdrome Pulmonary alveolar proteinosis Eosinophilic pneumonia... 

Hamada K, Teramoto S, Narita N, et al. Pulmonary veno-occlusive disease in pulmonary Langerhans cell granulomatosis. Eur Respir J 2000 15(2) 421-423. 

Histologically, these lesions correspond to UIP or NSIP. The administration of high doses of steroids may prevent the progression to fibrosis, although the evidence remains scarce. Combination therapy with vinca alkaloids has been associated with a more acute presentation, which histologically corresponds to DAD. The response to steroids is incomplete but may prevent progression to fibrosis. Diffuse alveolar hemorrhage and pulmonary veno-occlusive disease have also been reported (1). 

The pulmonary toxicity of busulfan was first reported in 1961. No clear risk factors for the development of lung toxicity have been consistently identified. The reported incidence of lung toxicity is 6% (4). The onset is typically subacute with fever and dry cough. Bleomycin is histologically characterized by NSIP or UIP. Pulmonary veno-occlusive disease and DIP have also been reported (1). Occasionally, busulfan may lead to alveolar proteinosis secondary to massive deposition of intracellular debris. This form of alveolar proteinosis is poorly responsive to whole lung lavage, and the role of steroids is not clearly estabhshed (9). 

Pulmonary veno-occlusive disease has also been reported with these agents (1). The role of corticosteroid therapy is unclear, though often proves ineffective. Cases of pulmonary fibrosis and pneumothoraces have rarely been reported with other nitrosourea agents (CCNU, methyl-CCNU, and DCNU). 

Izbicki G, Shitrit D, Schechtman 1, Bendayan D, Fink G, Sahar G, Saute M, Ben-Gal T, Kramer MR (2005) Recurrence of pulmonary veno-occlusive disease after heart-lung transplantation. J Heart Lung Transplant 24 635-637... 

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