Abstract
Pulmonary fibrosis (PF) is a very rare condition in children, which may be observed in specific forms of interstitial lung disease. None of the clinical, radiological, or histological descriptions used for PF diagnosis in adult patients, especially in situations of idiopathic PF, can apply to pediatric situations. This observation supports the view that PF expression may differ with age and, most likely, may cover distinct entities. The present review aims at summarizing the current understanding of PF pathophysiology in children and identifying suitable diagnostic criteria.
Highlights
In children, pulmonary fibrosis (PF) is a very rare condition, which has been sparsely described in specific forms of children’s interstitial lung disease. chILD has a reported incidence of 1–4 per millions of children and covers heterogeneous disorders in the immunocompetent host, such as surfactant disorders, pulmonary alveolar proteinosis (PAP), alveolar hemorrhage, neuroendocrine cell hyperplasia of infancy (NEHI), sarcoidosis, lung involvement of connective tissue diseases, hypersensitivity pneumonitis, and more than 25% of undefined chILD
usual interstitial pneumonia (UIP) is characterized on high resolution computed tomography (HRCT) scan by honeycombing together with traction bronchiectasis and a subpleural and lower lobe repartition of the lesions [7,8,9]
UIP is characterized by dense fibrosis with architectural distortion, predominant subpleural and/or paraseptal distribution of fibrosis, hyperplasic type 2 alveolar epithelial cells (AEC2), and fibroblastic foci with extracellular matrix (ECM) deposition in the absence of features suggesting an alternative diagnosis
Summary
Pulmonary fibrosis (PF) is a very rare condition, which has been sparsely described in specific forms of children’s interstitial lung disease (chILD). chILD has a reported incidence of 1–4 per millions of children and covers heterogeneous disorders in the immunocompetent host, such as surfactant disorders, pulmonary alveolar proteinosis (PAP), alveolar hemorrhage, neuroendocrine cell hyperplasia of infancy (NEHI), sarcoidosis, lung involvement of connective tissue diseases, hypersensitivity pneumonitis, and more than 25% of undefined chILD. UIP is characterized by dense fibrosis with architectural distortion, predominant subpleural and/or paraseptal distribution of fibrosis, hyperplasic type 2 alveolar epithelial cells (AEC2), and fibroblastic foci with extracellular matrix (ECM) deposition in the absence of features suggesting an alternative diagnosis. According to pediatric publications on chILD pathophysiology, lung fibrosis is a “destruction of pulmonary architecture caused by an abnormal wound repair response that leads to scar formation, organ malfunction, disruption of gas exchange, and respiratory failure” [12]. This definition mixes clinical, functional, and pathological issues of lung fibrosis and highlights pediatricians’ confusion over lung fibrosis’s definition. This review aims to explore the current knowledge on PF epidemiology, patterns, evolutive aspects through age, and natural history
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
