What is the prevalence of pulmonary veno-occlusive disease (PVOD)?

Updated: Oct 16, 2018
  • Author: Hakim Azfar Ali, MD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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In view of the underrecognition of pulmonary veno-occlusive disease (PVOD), the true incidence and prevalence of this condition is unknown. Many cases are treated as idiopathic pulmonary arterial hypertension (PAH). Estimates indicate, however, that 6% of patients clinically believed to have primary pulmonary hypertension may have evidence of PVOD at autopsy. [26] Depending on the cited series, the estimated prevalence of PVOD among patients clinically diagnosed with idiopathic PAH varies from 5-20%. [2]

An annual incidence of PVOD of 0.1-0.2 case per million persons in the general population has been suggested based on the incidence of primary pulmonary hypertension in the general population. [27, 28] Note, however, that such indirect estimates of a rare disease’s incidence may be imprecise.

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