Rare clinical case: pulmonary venoocclusive disease

Pulmonary veno-occlusive disease (PVOD) is a rare pathology of unknown etiology when the development and progression of pulmonary arterial hypertension (PAH) is associated with damage to small pulmonary veins and venules, including thrombotic lesions. The prevalence of the disease is 1 – 2 cases per 10 million people. PVOD is characterized by rapid progression and poor prognosis. The aim of the work was to demonstrate a rare clinical case of PVOD in a 43-year-old woman who had a predisposing risk factor for the development of the disease and a verified mutation in the EIF2AK4 gene. Conclusion. This clinical case illustrates the difficulties of diagnosing PVOD and choosing the further patient management. It is noted that timely diagnosis and initiation of therapy are critically important for patients with PVOD, while the use of PAH-specific therapy might complicate course of the disease by provoking pulmonary edema.