lung disease

Interstitial lung disease

Interstitial lung disease (ILD), also known as diffuse parenchymal lung disease (DPLD), refers to a group of lung diseases affecting the interstitium of the lung: alveolar epithelium, pulmonary capillary endothelium, basement membrane, perivascular and perilymphatic tissues. The term ILD is used to distinguish these diseases from obstructive airways diseases.

Most types of ILD involve fibrosis, but this is not essential; indeed fibrosis is often a later feature. The phrase "pulmonary fibrosis" is no longer considered a synonym, but the term is still used to denote ILD involving fibrosis. The term is commonly combined with idiopathic in "idiopathic pulmonary fibrosis", denoting fibrotic ILD that cannot be ascribed to a distinct primary cause.

Causes

ILD may be classified according to the cause. One method of classification is as follows

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

Investigation

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.

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, such as prednisolone. Some patients respond to immunosuppressant treatment. Patients with hypoxemia may be given supplemental oxygen.

References

External links

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