Pulmonary Veno-Occlusive Disease (PVOD) Associated with Connective Tissue Disease

Pulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD) has a worse prognosis than that of idiopathic PAH, even when drugs for PAH treatment, such as vasodilators, are used. In cases of CTD-PAH, if pulmonary veins or venules are also affected, the condition is termed pulmonary veno-occlusive disease (PVOD). PVOD is thought to worsen the prognosis of CTD-PAH. Although surgical biopsy is required for a definitive diagnosis of PVOD. findings of chest computed tomography (CT) or the response to PAH drugs may facilitate diagnosis. There is no effective treatment for PVOD except lung transplantation. However, immunosuppressive therapy may be one treatment choice for PVOD associated with CTD. Treatment strategies should be "tailor-made" for each patient since CTD patients usually have multiple-organ involvement.