What are the immune-mediated causes 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

Either idiopathic autoimmune injury to venules or immune-mediated injury to venules related to viral or other environmental agents has been proposed as a mechanism for the development of PVOD. Cases have been reported in patients with other autoimmune clinical features. [10, 11]

Some cases of pulmonary hypertension in the setting of mixed connective disease and scleroderma, including the CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) variant, have been known to have a histopathology consistent with that of PVOD. [12, 13, 14]


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