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. 2022 Mar 21;11(6):1747.
doi: 10.3390/jcm11061747.

Etiologic Classification of Diffuse Parenchymal (Interstitial) Lung Diseases

Matthias Griese  1
Affiliations

Etiologic Classification of Diffuse Parenchymal (Interstitial) Lung Diseases

Matthias Griese. J Clin Med. .

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, exposure-related 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.

Keywords: categorization; children’s interstitial lung disease (chILD); classification; familial; human phenotype ontology; idiopathic interstitial fibrosis; interstitial pneumonia; interstitial pneumonitis; surfactant.

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Conflict of interest statement

M. Griese has been provided paid services in clinical study development to Böhringer Ingelheim, Germany. and Roche, UK.

Figures

Figure 1
Figure 1
Overview of interstitial (diffuse parenchymal) lung diseases and their neighbors. Diffuse lung diseases involve the entire lungs, i.e., from base to top, peripheral to central, and front to back. Many airway disorders, including COPD, asthma, and cystic fibrosis, can be diffuse, whereas others remain more localized; few pleural diseases, gross structural abnormalities such as congenital pulmonary malformation type 0, or pleural disorders are diffuse. The numerically largest group of diffuse lung diseases are the interstitial (diffuse parenchymal) lung diseases (ILD). These consist of four large etiologic categories of diseases, affecting the lung interstitium or parenchyma, (1) the lung-only (native parenchymal) disorders, (2) the systemic disease-related disorders, (3) exposure-related disorders, and (4) the vascular disorders. ILDs from all age groups are included.
Figure 2
Figure 2
Diagnostic algorithm for the identification and etiologic classification of ILD.
Figure 3
Figure 3
Etiologic disease classification of ILDs drives diagnostic and treatment opportunities. The integration of more and more disease-related data from deeper analysis and systems’ medicine will result in personalized medicine, allowing more precise diagnosis and treatment.
See this image and copyright information in PMC

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