The disease is progressive and fatal, with median survival of about 2 years from the time of diagnosis to death.

Though this does not mean other possible causes do not exist, such as viral infection and risk of toxic chemicals (chemotherapy drugs).

Published reports have indicated fatal occurrences that appeared to possess a familial pattern, more to the point, a germline mutation.

[7] The pathophysiology of veno-occlusive disease culminates in occlusion of the pulmonary blood vessels.

Thickening is identified in lobular septal veins, also dilatation of lymphatics happens.