Pulmonary veno-occlusive disease (PVOD) is a rare and serious form of pulmonary hypertension. Management depends on identifying the underlying aetiology — whether idiopathic, heritable, or associated with connective tissue disease — as each may carry distinct therapeutic implications.
Reports indicate that immunosuppressive therapy may be of benefit in patients with idiopathic, heritable, or connective tissue disease-associated PVOD. The full structured regimen — including specific agents, sequencing, and clinical guidance — is available in the complete protocol.
There were some reports of the benefit of immunosuppressive agents in patients with idiopathic and heritable PVOD and patients with connective tissue disease-associated PVOD.
This is thought to be from alleviating the inflammatory process implicated in PVOD.
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