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Pulmonary hypertension (PH) is a severe health condition in which the mean pulmonary arterial pressure increases to 25 mmHg or more, even when the body is at rest. This high pressure in the blood vessels that transport blood from the heart to the lungs can cause various symptoms, including shortness of breath, can lead to right heart failure, and significantly affect the overall quality of life.
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Related Experiment Video

Updated: Sep 29, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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Etiologic Classification of Diffuse Parenchymal (Interstitial) Lung Diseases.

Matthias Griese1

  • 1Department of Pediatric Pneumology, Dr. von Haunersches Kinderspital, University of Munich, German Center for Lung Research, Lindwurmstr. 4a, D-80337 Munich, Germany.

Journal of Clinical Medicine
|March 25, 2022
PubMed
Summary

This study reviews interstitial lung diseases (ILD) classification, proposing an etiologic approach. This framework aids personalized treatments and research for rare lung disorders.

Keywords:
categorizationchildren’s interstitial lung disease (chILD)classificationfamilialhuman phenotype ontologyidiopathic interstitial fibrosisinterstitial pneumoniainterstitial pneumonitissurfactant

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Area of Science:

  • Pulmonology and Respiratory Medicine
  • Medical Classification Systems
  • Genetics and Genomics in Disease

Background:

  • Interstitial lung diseases (ILD), also known as diffuse parenchymal lung diseases (DPLD), encompass a wide spectrum of rare and common respiratory disorders.
  • Accurate classification is crucial for understanding disease mechanisms, genetic and environmental risk factors, and developing personalized treatment strategies.
  • Existing classification systems often lack a unified approach, particularly in integrating pediatric and adult cases and leveraging molecular data.

Purpose of the Study:

  • To present a historical overview of ILD classification systems in adult and pediatric pneumology.
  • To propose and advocate for an etiologic classification framework for ILD.
  • To highlight the importance of molecularly defined entities for precision medicine and the integration of -omics data in understanding idiopathic lung diseases.

Main Methods:

  • Review of historical ILD classification systems.
  • Development of a proposed etiologic classification with four main categories: lung-only, systemic disease-related, exposure-related, and vascular disorders.
  • Consideration of data from the European children's ILD register (chILD-EU) to inform clinical practice and registry work.

Main Results:

  • The proposed etiologic classification integrates adult and pediatric lung diseases into a single system.
  • It categorizes ILDs into lung-only, systemic disease-related, exposure-related, and vascular disorders.
  • Genomics and other -omics data are identified as central to advancing the understanding and treatment of idiopathic lung diseases.

Conclusions:

  • An etiologic classification system offers a unified approach for both clinical practice and research in ILD.
  • Splitting diseases into molecularly defined entities is essential for precision medicine.
  • A systems' medicine approach integrating diverse data is the future direction for advancing ILD care and bringing precision medicine to patients.