Abandoning developmental silos: what can paediatricians and adult interstitial lung disease physicians learn from each other?

Current Opinion in Pulmonary Medicine
Paolo SpagnoloAndrew Bush

Abstract

Interstitial lung disease (ILD) consists of a large and heterogeneous group of disorders that are classified together because of similar clinical, radiographic, physiologic or pathologic manifestations. Overall, although there is overlap between adult and childhood ILD (chILD), there are many differences in disease causes and prevalences. In the last few years, our understanding of adult ILD pathobiology has improved substantially. This is particularly true for idiopathic pulmonary fibrosis, the most common of the idiopathic interstitial pneumonias, wherein recently developed guideline documents provide recommendations for the diagnosis and clinical management of patients. For chILD, similar guidelines are yet to be developed. However, complications and long-term pulmonary outcomes of paediatric disease are better appreciated, which make the implementation of a successful transition program from paediatric to adult care an urgent need. Similarly important is the development of guidelines on performance and interpretation of genetic testing in affected and unaffected relatives of familial cases and in children of adult-onset ILD patients. Lung transplantation appears to be as successful as in adult patients for end-stage disease...Continue Reading

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