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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Interstitial lung disease in children
Shailendra Das1, Claire Langston, Leland L Fan
1Pediatric Pulmonary Section, Department of Pediatrics, USA.
Insights
Interstitial lung disease (ILD) in children is rare, with unique causes and presentations in infancy. Diagnosis relies on clinical, radiologic, and pathologic findings, with newer methods reducing the need for lung biopsy.
Area of Science:
- Pediatric Pulmonology
- Rare Diseases
- Interstitial Lung Diseases
Background:
- Interstitial lung diseases (ILDs) in children are rare, diffuse lung diseases with diverse etiologies.
- Causes include genetic factors, systemic diseases, and inflammatory or fibrotic responses.
- Unique presentations and causes are observed in infancy.
Purpose of the Study:
- To review recent advances in understanding pediatric interstitial lung disease (ILD).
- To cover the cause, pathogenesis, presentation, diagnosis, treatment, and prognosis of childhood ILD.
Main Methods:
- Review of current literature on pediatric ILD.
- Discussion of classification schemes for ILD in children.
- Evaluation of diagnostic approaches, including noninvasive and invasive studies.
Main Results:
- ILD classification in children >2 years often uses adult schemes; pathological evaluation is key for <2 years.
- Pathological categories include developmental, growth, and surfactant dysfunction disorders.
- Neuroendocrine cell hyperplasia of infancy and pulmonary interstitial glycogenosis have favorable prognoses.
Conclusions:
- Pediatric ILDs stem from various pathogenic processes, with distinct infantile forms.
- Diagnosis integrates clinical, radiologic, and pathological findings.
- Ongoing research aims to minimize the necessity of lung biopsies.
Purpose Of Review:
In this review, we discuss the recent advances in our understanding of the cause, pathogenesis, presentation, diagnosis, treatment, and prognosis of interstitial lung disease (ILD) in children.
Recent Findings:
The classification of ILD syndromes in children greater than 2 years of age is based largely on adult classification schemes. In children less than 2 years of age, classification has been developed and evaluated pathologically. Entities can be categorized into developmental disorders, growth abnormalities, and surfactant dysfunction disorders based on pathologic findings. Two distinctive entities, neuroendocrine cell hyperplasia of infancy and pulmonary interstitial glycogenosis, present early in life with characteristic findings. These two disorders appear to have a favorable prognosis. Diagnosis of ILD syndromes is based on the summation of history and physical findings and both noninvasive and invasive studies. Newer approaches are being evaluated to decrease the need for lung biopsy.
Summary:
Children's interstitial lung diseases are rare diffuse lung diseases resulting from a variety of pathogenic processes that include genetic factors, association with systemic disease processes, and inflammatory or fibrotic responses to stimuli. There are unique causes and presentations seen in infancy. Diagnosis in these disorders is made by the summation of clinical, radiologic, and pathologic findings.
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