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Interstitial lung disease
Classification and external resources

End-stage pulmonary fibrosis of unknown origin, taken from an autopsy in the 1980s.
ICD-10 J84.9
ICD-9 506.4, 508.1, 515, 516.3, 714.81, 770.7
DiseasesDB 31509
eMedicine ped/1950
MeSH D017563

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".

Contents

Causes

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:

  1. Inhaled substances
  2. Drug induced
  3. Connective tissue disease
  4. Infection
  5. Idiopathic
  6. Malignancy

Investigation

Patients with pneumocystis pneumonia can present with interstitial lung disease, as seen in the reticular markings on this AP chest x-ray

Investigation is tailored towards the symptoms and signs. Most patients have blood testing, chest x-ray, pulmonary function testing, and high resolution CT thorax.

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

TLCO will be decreased in these patients.

Treatment

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

External links

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

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