Rare clinical case: pulmonary venoocclusive disease
https://doi.org/10.18093/0869-0189-2024-34-4-595-598
Abstract
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.
About the Authors
V. A. Stener
Federal State Autonomous Educational Institution of Higher Education I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University)
Russian Federation
Competing Interests:
Russian Federation
Valeriya A. Stener – 5th year student, N.V.Sklifosovsky Institute of Clinical Medicine, Federal State Autonomous Educational Institution of Higher Education I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University).
Trubetskaya ul. 8, build. 2, Moscow, 119991
Tel.: (499) 201-21-01
Competing Interests:
The author report that there is no conflict of interest.
N. A. Tsareva
Federal State Autonomous Educational Institution of Higher Education I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University); Federal State Budgetary Institution “Pulmonology Scientific Research Institute” under Federal Medical and Biological Agency of Russian Federation
Russian Federation
Competing Interests:
Russian Federation
Natalya A. Tsareva - Candidate of Medicine, Associate Professor, Department of Pulmonology, N.V. Sklifosovsky Institute of Clinical Medicine, Federal State Autonomous Educational Institution of Higher Education I.M.Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University); Head of the Laboratory of Intensive Care and Respiratory Failure, Federal State Budgetary Institution “Pulmonology Scientific Research Institute” under Federal Medical and Biological Agency of Russian Federation.
Trubetskaya ul. 8, build. 2, Moscow, 119991; Orekhovyy bul’var 28, Moscow, 115682
Tel.: (495) 609-14-00
Competing Interests:
The author report that there is no conflict of interest.
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For citations:
Stener V.A., Tsareva N.A. Rare clinical case: pulmonary venoocclusive disease. PULMONOLOGIYA. 2024;34(4):595-598. (In Russ.) https://doi.org/10.18093/0869-0189-2024-34-4-595-598
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