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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Published on: June 16, 2020

Chronic interstitial lung disease in children.

Matthias Griese¹

¹Hauner Children's Hospital, University of Munich, German Center for Lung Research, Munich, Germany matthias.griese@med.uni-muenchen.de.

European Respiratory Review : an Official Journal of the European Respiratory Society

|February 14, 2018

Summary

Children's interstitial lung diseases (chILD) are increasingly recognized. Research highlights prevalence, genetic links, and treatment advancements, emphasizing collaborative care between pediatric and adult specialists for better outcomes.

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Area of Science:

Pediatric Pulmonology
Rare Respiratory Diseases
Genetic Lung Disorders

Background:

Children's interstitial lung diseases (chILD) encompass diverse genetic and developmental lung disorders.
Global registries like ARNOLD and chILD-EU are crucial for understanding chILD prevalence and management.
Recent research advances understanding of specific chILD types, including those linked to genetic mutations.

Purpose of the Study:

To review recent publications and progress in pediatric interstitial lung disease.
To highlight challenges and advancements in chILD research and clinical practice.
To emphasize the need for collaboration between pediatric and adult pulmonologists.

Main Methods:

Review of publications presented at the 2017 European Respiratory Society (ERS) Annual Congress.
Analysis of data from international chILD registries (e.g., ARNOLD, chILD-EU).
Synthesis of findings from cohort studies and translational research.

Main Results:

Period prevalence of chILD in immunocompetent children estimated at 1.5 cases per million, with a 7% mortality rate.
Identification of genetic links, such as ABCA3 mutations, contributing to chILD across age groups.
Successful lung transplantation outcomes reported for surfactant dysfunction disorders in children.

Conclusions:

Progress in chILD research necessitates continued registry efforts and translational studies.
Effective management requires standardized diagnostic and treatment protocols.
Enhanced collaboration between pediatric and adult pulmonologists is vital for advancing the field of chILD.