Interstitial lung diseases J84.9

Last updated on: 09.11.2024

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Definition
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Interstitial lung diseases (also known as parenchymal lung diseases) are a heterogeneous group of pulmonary diseases characterized by thickening of the alveolar septa, fibroblast proliferation, collagen deposition and, if the disease progresses unhindered, pulmonary fibrosis. Interstitial lung diseases can be classified according to various criteria (e.g. acute versus chronic, granulomatous versus non-granulomatous, known cause versus unknown cause, primary lung disease versus secondary consequence of systemic disease, history of smoking versus history of non-smoking).

Classification
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A) Known causes (50 % of cases):

B) Unknown causes

  • Idiopathic interstitial pneumonia (IIP) Prevalence: approx. 70 (f) or 80 (m)/100,000. Classification of IIP (ATS = American Thoracic Society and ERS = European Respiratory Society, 2013) -
  • 1. main forms of idiopathic interstitial pneumonia (major IIPs)
  • Chronic fibrosing IIPs
  • Idiopathic pulmonary fibrosis (IPF, 55%)
  • Non-specific interstitial pneumonia (NSIP, 25%) 95%
  • Tobacco smoke-associated IIPs (SR-ILD, 15%)
  • Acute/subacute IIPs
    • Cryptogenic organizing pneumonia (COP, 3%)
    • Acute interstitial pneumonia (AIP, 1%)
  • 2.rare idiopathic interstitial pneumonia (rare IIPs)
    • Lymphocytic interstitial pneumonia (LIP, <1%)
    • Pleuroparenchymal fibroelastosis (PPFE)
  • 3. unclassifiable idiopathic interstitial pneumonia (unclassifiable IIPs)

Occurrence/Epidemiology
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Incidence: 10 - 25/100 000/year.

Manifestation
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The term "interstitial lung disease" covers more than 300 different diseases. Most of them are rare. The most common interstitial lung diseases include:

  • Sarcoidosis
  • Idiopathic pulmonary fibrosis
  • Extrinsic allergic alveolitis (also known as hypersensitive pneumonitis)
  • Interstitial lung diseases associated with tissue diseases
  • Pneumoconiosis
  • Interstitial lung diseases caused by certain drugs used to treat other diseases
  • Some interstitial lung diseases, especially sarcoidosis, also affect other organs of the body.

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

Last updated on: 09.11.2024