Interstitial lung disease

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Interstitial lung disease
Classification and external resources
End-stage interstitial lung disease (honeycomb lung).jpg
End-stage pulmonary fibrosis of unknown origin, taken from an autopsy in the 1980s.
ICD-10J84.9
ICD-9506.4, 508.1, 515, 516.3, 714.81, 770.7
 
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Interstitial lung disease
Classification and external resources
End-stage interstitial lung disease (honeycomb lung).jpg
End-stage pulmonary fibrosis of unknown origin, taken from an autopsy in the 1980s.
ICD-10J84.9
ICD-9506.4, 508.1, 515, 516.3, 714.81, 770.7

Interstitial lung disease (ILD), also known as diffuse parenchymal lung disease (DPLD),[1] refers to a group of lung diseases affecting the interstitium (the tissue and space around the air sacs of the lungs). [2] It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, perivascular and perilymphatic tissues.

The term ILD is used to distinguish these diseases from obstructive airways diseases.

Prolonged ILD may result in pulmonary fibrosis, but this is not always the case. Idiopathic pulmonary fibrosis is one form of "interstitial lung disease".

Causes[edit]

The alveoli
Micrograph of usual interstitial pneumonia (UIP). UIP is the most common pattern of idiopathic interstitial pneumonia (a type of interstitial lung disease) and usually represents idiopathic pulmonary fibrosis. H&E stain. Autopsy specimen.

ILD may be classified according to the cause.[3] One method of classification is as follows: (Mnemonic- Inhaled Drugs Can Infect Idiopathic Malignancy)

  1. Inhaled substances
  2. Drug induced
  3. Connective tissue disease
  4. Infection (mnemonic- TB is CRAP)
  5. Idiopathic
  6. Malignancy

Investigation[edit]

Patients with pneumocystis pneumonia can present with interstitial lung disease, as seen in the reticular markings on this AP chest x-ray
A chest X-ray demonstrating pulmonary fibrosis due to amiodarone.

Investigation is tailored towards the symptoms and signs. Most patients have blood testing, chest x-ray, pulmonary function testing, and high resolution CT thorax. HRCT thorax is the best mode of investigation to diagnose interstitial lung disease.

A lung biopsy is required if the clinical history and imaging are not clearly suggestive of a specific diagnosis or malignancy cannot otherwise be ruled out.

DLCO will be decreased in these patients.

Treatment[edit]

ILD is not a single disease, but encompasses many different pathological processes. Hence treatment is different for each disease.

If a specific occupational exposure cause is found, the person should avoid that environment. If a drug cause is suspected, that drug should be discontinued.

Many idiopathic and connective tissue-based causes of ILD are treated with corticosteroids,[4] such as prednisolone. Some patients respond to immunosuppressant treatment. Patients with hypoxemia may be given supplemental oxygen.

References[edit]

External links[edit]

For more information and resources on ILD, please visit the UCSF ILD Program website