Description
Title: Pathogenesis, risk factors, clinical characteristics, and the most recent diagnostic algorithm for pulmonary veno-occlusive disease
Abstract: The estimated prevalence of pulmonary veno-occlusive disease (PVOD) and pulmonary capillary haemangiomatosis (PCH), two rare diseases, is less than 1 case per million people. Pre-septal and septal veins, alveolar capillaries, and small pulmonary arteries are all affected by the vascular pathology in PVOD/PCH. PVOD/PCH has been classified as belonging to subgroup 1′ of pulmonary arterial hypertension by the ERS/ESC classification of pulmonary hypertension (PH) from 2015 (PAH). However, recent data point to the possibility of PVOD/PCH pathology in patients with group 1 diagnoses. The issue may be with scleroderma-related PAH, drug-induced PAH, HIV-related PAH, and up to 10% of patients with idiopathic PAH (IPAH). Bi-allelic EIF2AK4 mutations have recently been identified in 9% of sporadic PVOD/PCH cases as well as in cases with the heritable form of the disease. Furthermore, it was established a link between occupational exposure to organic solvents and PVOD/PCH. The current review aims to condense the most recent information on PVOD/pathogenesis, PCH’s risk factors, clinical characteristics, and diagnostic algorithm.
Keywords: pulmonary venoocclusive disease; risk factors; genetic dependence
Paper Quality: SCOPUS / Web of Science Level Research Paper
Subject: Advances in Respiratory Medicine
Writer Experience: 20+ Years
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