Which conditions should be considered in the differential diagnosis 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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Answer

In a patient with significant pulmonary hypertension, the possibility of chronic pulmonary thromboembolic disease must be considered because it is one of the common and treatable causes of pulmonary hypertension. More common causes of pulmonary venous hypertension (eg, systolic or diastolic left-sided heart failure) and valvular heart disease (eg, mitral stenosis) should be considered as a part of the differential diagnosis before a diagnosis of pulmonary veno-occlusive disease (PVOD) is established.

Vascular remodeling similar to PVOD occurs in both pulmonary veins and arteries in conditions such as mitral stenosis and fibrosing mediastinitis. However, the structural changes in the veins are more prominent in PVOD than in these other two conditions and may therefore provide a morphologic approach to differentiation of these diseases. [25]

In some cases of PVOD, advanced parenchymal lung diseases (eg, sarcoidosis, interstitial lung disease, pneumoconioses) may be considered as diagnostic possibilities because of prominent interstitial changes seen on chest radiographs. However, the distinction between most forms of advanced interstitial lung disease from PVOD is relatively straightforward for most experts upon review of chest CT scans. Pulmonary capillary hemangiomatosis should also be considered in the differential diagnosis of PVOD.


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