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Pulmonary capillary haemangiomatosis: a distinct entity?
- Source :
- European Respiratory Review, Vol 29, Iss 156 (2020)
- Publication Year :
- 2020
- Publisher :
- European Respiratory Society, 2020.
-
Abstract
- Pulmonary capillary haemangiomatosis (PCH) is a rare and incompletely understood histopathological finding characterised by abnormal capillary proliferation within the alveolar interstitium, which has long been noted to share many overlapping features with pulmonary veno-occlusive disease (PVOD). But are PCH and PVOD distinct entities that occur in isolation, or are they closely intertwined manifestations along a spectrum of the same disease? The classic clinical features of both PCH and PVOD include signs and symptoms related to pulmonary hypertension, hypoxaemia, markedly impaired diffusion capacity of the lung and abnormal chest imaging with ground glass opacities, septal lines and lymphadenopathy. In recent years, increasing evidence suggests that the clinical presentation, histopathological features, genetic substrate and pathobiological mechanisms of PCH and PVOD are overlapping and usually indistinguishable. The discovery of biallelic mutations in the eukaryotic translation initiation factor 2 α kinase 4 (EIF2AK4) gene in heritable PCH and PVOD greatly advanced our understanding of the overlapping nature of these conditions. Furthermore, recognition of PCH and PVOD-like changes in other pulmonary vascular diseases and in conditions that cause chronic pulmonary venous hyper-perfusion or hypertension suggests that PCH/PVOD may develop as a reactive process to various insults or injuries to the pulmonary vasculature, rather than being primary angiogenic disorders.
- Subjects :
- Pulmonary and Respiratory Medicine
Abnormal chest
Pathology
medicine.medical_specialty
Lung Neoplasms
Signs and symptoms
Disease
Risk Assessment
Pulmonary capillary haemangiomatosis
Risk Factors
Terminology as Topic
Humans
Medicine
Genetic Predisposition to Disease
Hemangioma, Capillary
lcsh:RC705-779
Pulmonary Arterial Hypertension
Lung
business.industry
lcsh:Diseases of the respiratory system
Prognosis
medicine.disease
Pulmonary hypertension
Capillaries
Pulmonary Alveoli
Phenotype
medicine.anatomical_structure
Pulmonary Veins
Mutation
Pulmonary Veno-Occlusive Disease
Pulmonary vasculature
business
Subjects
Details
- Language :
- English
- ISSN :
- 16000617 and 09059180
- Volume :
- 29
- Issue :
- 156
- Database :
- OpenAIRE
- Journal :
- European Respiratory Review
- Accession number :
- edsair.doi.dedup.....2565a69dd4c76ac4ab4edf7d2850da01