Pulmonary veno-occlusive disease

Pulmonary veno-occlusive disease is a rare disease.
Its prevalence is estimated to be 0.1 to 0.2 cases per million population, although it is likely that many cases are unrecognized.
Mandel J, Mark EJ, Hales CA. Pulmonary veno-occlusive disease. Am J Respir Crit Care Med 2000;162:1964-73.

The cause of the disease remains obscure. Mutations in the gene encoding bone morphogenetic protein receptor
type 2 (BMPR2) (described in some cases of idiopathic pulmonary arterial hypertension) have been reported in some patients with pulmonary veno-occlusive disease.4 The disease has also been described in patients with scleroderma5; in such cases, the results of radiographic testing can be
confusing, since interstitial markings from pulmonary
edema may be erroneously attributed to parenchymal fibrosis. One recent report described the occurrence of pulmonary veno-occlusive disease in a patient who reported use of anorexigens,
although anorexigen use has been more characteristically
linked to pulmonary arterial hypertension

CT findings in patients with pulmonary venoocclusive
disease include smooth interlobular
septal thickening, centrilobular ground-glass opacities,
pleural effusions, enlarged pulmonary arteries,
small or normal-size pulmonary veins, and
adenopathy. In a study comparing CT findings in
pulmonary veno-occlusive disease with those in
pulmonary arterial hypertension, ground-glass
opacities with a centrilobular pattern and septal
thickening were more consistent with pulmonary
veno-occlusive disease; adenopathy had a high
specificity but a low sensitivity for the condition

However, the triad of pulmonary edema, normal left ventricular enddiastolic
pressure (in conjunction with severe pulmonary hypertension), and the CT findings (ground-glass opacities, septal lines, and adenopathy) strongly suggested pulmonary veno-occlusive disease.