Pulmonary veno-occlusive disease in Sjogren's syndrome: a case report
China: The researchers from the Cardiology department of Xiangya Hospital have presented the first report mentioning patients with Pulmonary Veno-occlusive disease (PVOD) associated SS-PAH (Sjoger Syndrome-Pulmonary arterial hypertension) carrying novel EIF2AK4 mutation, responded well to PAH-targeted therapies.
The researchers highly pointed location of the EIF2AK4 mutation may be responsible for the different responses to targeted therapy and disease phenotypes.
The case report is published in BMC Pulmonary Medicine.
It is already known that Pulmonary arterial hypertension or PAH is associated with connective tissue disease (CTD) belongs to Group 1 pulmonary hypertension.
PVOD, characterized by venous system aberrations, has been previously reported in CTD-PAH, but its association with SS (Sjoger's Syndrome) has rarely been reported.
Zeng et al. and colleagues presented this rare case report.
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