Pulmonary veno-occlusive disease: pathogenesis, risk factors, clinical features and diagnostic algorithm - state of the art.

Monika Szturmowicz,Jan Kuś,Ewa Szczepulska,Małgorzata Szołkowska,Barbara Burakowska,Aneta Kacprzak

doi:10.5603/arm.2018.0021

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.

Highlights

Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary haemangiomatosis (PCH) constitute the subgroup 1’ of pulmonary arterial hypertension (PAH) in the clinical classification of pulmonary hypertension (PH) (Table 1) [1]
PVOD/ PCH is believed to be an unrecognised cause of pulmonary hypertension in up to 10% of patients diagnosed with idiopathic PAH (IPAH) [1, 3]
Findings on lung High resolution computed tomography of the chest (HRCT) in PVOD/PCH have to be differentiated from interstitial lung disease

Summary

Introduction

Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary haemangiomatosis (PCH) constitute the subgroup 1’ of pulmonary arterial hypertension (PAH) in the clinical classification of pulmonary hypertension (PH) (Table 1) [1]. PVOD/ PCH is believed to be an unrecognised cause of pulmonary hypertension in up to 10% of patients diagnosed with idiopathic PAH (IPAH) [1, 3]. PVOD/PCH was reported in a patient with diffuse lung emphysema and severe pulmonary hypertension [10].

Results

Conclusion