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Griese, Matthias ORCID: 0000-0003-0113-912X (2022): Etiologic Classification of Diffuse Parenchymal (Interstitial) Lung Diseases. Journal of Clinical Medicine, 11: 1747. pp. 1-19. ISSN 2077-0383

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Abstract

Interstitial lung diseases (ILD) or diffuse parenchymal lung diseases (DPLD) comprise a large number of disorders. Disease definition and classification allow advanced and personalized judgements on clinical disease, risks for genetic or environmental transmissions, and precision medicine treatments. Registers collect specific rare entities and use ontologies for a precise description of complex phenotypes. Here we present a brief history of ILD classification systems
from adult and pediatric pneumology. We center on an etiologic classification, with four main categories: lung‐only (native parenchymal) disorders, systemic disease‐related disorders, exposurerelated disorders, and vascular disorders. Splitting diseases into molecularly defined entities is key for precision medicine and the identification of novel entities. Lumping diseases targeted by similar
diagnostic or therapeutic principles is key for clinical practice and register work, as our experience with the European children’s ILD register (chILD‐EU) demonstrates. The etiologic classification
favored combines pediatric and adult lung diseases in a single system and considers genomics and other ‐omics as central steps towards the solution of “idiopathic” lung diseases. Future tasks focus
on a systems’ medicine approach integrating all data and bringing precision medicine closer to the patients.

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