Next Article in Journal
Immune Checkpoint Inhibitors in Non-Small Cell Lung Cancer—Towards Daily Practice
Previous Article in Journal
Difficulties in the Treatment of Recurring Diffuse Alveolar Hemorrhage Accompanying Primary Antiphospholipid Syndrome: A Case Report and Literature Review
 
 
Search for Articles:
Title / Keyword
Author / Affiliation / Email
Journal
Article Type
 
 
Advanced Search
 
Section
Special Issue
Volume
Issue
Number
Page
 
Journals
ARM
Volume 86
Issue 3
10.5603/ARM.2018.0021
arm-logo
Submit to this Journal Review for this Journal Propose a Special Issue
► Article Menu

Article Menu

share Share announcement Help format_quote Cite question_answer Discuss in SciProfiles
Advances in Respiratory Medicine is published by MDPI from Volume 90 Issue 4 (2022). Previous articles were published by another publisher in Open Access under a CC-BY (or CC-BY-NC-ND) licence, and they are hosted by MDPI on mdpi.com as a courtesy and upon agreement with Via Medica.
first_page
settings
Order Article Reprints
Font Type:
Arial Georgia Verdana
Font Size:
Aa Aa Aa
Line Spacing:
Column Width:
Background:
Review

Pulmonary Veno-Occlusive Disease: Pathogenesis, Risk Factors, Clinical Features and Diagnostic Algorithm—State of the Art

by
Monika Szturmowicz
1,*,
Aneta Kacprzak
1,
Małgorzata Szołkowska
2,
Barbara Burakowska
3,
Ewa Szczepulska
2 and
Jan Kuś
1
1
1st Department of Lung Diseases, National Tuberculosis and Lung Diseases Research Institute, Warsaw, Poland
2
Department of Pathology, National Tuberculosis and Lung Diseases Research Institute, Warsaw, Poland
3
Department of Radiology, National Tuberculosis and Lung Diseases Research Institute, Warsaw, Poland
*
Author to whom correspondence should be addressed.
Adv. Respir. Med. 2018, 86(3), 131-141; https://doi.org/10.5603/ARM.2018.0021
Submission received: 19 March 2018 / Revised: 29 May 2018 / Accepted: 29 May 2018 / Published: 30 June 2018

Abstract

Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary haemangiomatosis (PCH) are rare disorders, with the estimated prevalence of less than 1 case per million inhabitants. The vascular pathology in PVOD/PCH involves pre-septal and septal veins, alveolar capillaries and small pulmonary arteries. According to the ERS/ESC classification of pulmonary hypertension (PH) from 2015, PVOD/PCH have been included in the subgroup 1’ of pulmonary arterial hypertension (PAH). Recent data indicate, however, the possibility of PVOD/PCH pathology in the patients diagnosed in the group 1. The problem may concern PAH associated with scleroderma, drug- induced PAH, PAH due to HIV infection and up to 10% of patients with idiopathic PAH (IPAH). Recently, bi-allelic EIF2AK4 mutations were found in the cases with heritable form of PVOD/PCH and in about 9% of sporadic cases. Moreover, an association between occupational exposure to organic solvents and PVOD/PCH was proved. The present review is an attempt to summarise the current data on pathogenesis, risk factors, clinical features and diagnostic algorithm for PVOD/PCH.
Keywords: pulmonary venoocclusive disease; risk factors; genetic dependence pulmonary venoocclusive disease; risk factors; genetic dependence

Share and Cite

MDPI and ACS Style

Szturmowicz, M.; Kacprzak, A.; Szołkowska, M.; Burakowska, B.; Szczepulska, E.; Kuś, J. Pulmonary Veno-Occlusive Disease: Pathogenesis, Risk Factors, Clinical Features and Diagnostic Algorithm—State of the Art. Adv. Respir. Med. 2018, 86, 131-141. https://doi.org/10.5603/ARM.2018.0021

AMA Style

Szturmowicz M, Kacprzak A, Szołkowska M, Burakowska B, Szczepulska E, Kuś J. Pulmonary Veno-Occlusive Disease: Pathogenesis, Risk Factors, Clinical Features and Diagnostic Algorithm—State of the Art. Advances in Respiratory Medicine. 2018; 86(3):131-141. https://doi.org/10.5603/ARM.2018.0021

Chicago/Turabian Style

Szturmowicz, Monika, Aneta Kacprzak, Małgorzata Szołkowska, Barbara Burakowska, Ewa Szczepulska, and Jan Kuś. 2018. "Pulmonary Veno-Occlusive Disease: Pathogenesis, Risk Factors, Clinical Features and Diagnostic Algorithm—State of the Art" Advances in Respiratory Medicine 86, no. 3: 131-141. https://doi.org/10.5603/ARM.2018.0021

APA Style

Szturmowicz, M., Kacprzak, A., Szołkowska, M., Burakowska, B., Szczepulska, E., & Kuś, J. (2018). Pulmonary Veno-Occlusive Disease: Pathogenesis, Risk Factors, Clinical Features and Diagnostic Algorithm—State of the Art. Advances in Respiratory Medicine, 86(3), 131-141. https://doi.org/10.5603/ARM.2018.0021

Article Metrics

Back to TopTop