| Interstitial lung disease | |
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| Classification and external resources | |
End-stage pulmonary fibrosis of unknown origin, taken from an autopsy in the 1980s. |
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| ICD-10 | J84.9 |
| ICD-9 | 506.4, 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".
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ILD may be classified according to the cause.[3] One method of classification is as follows:
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 are not clearly suggestive of a specific diagnosis or malignancy cannot otherwise be ruled out.
TLCO will be decreased in these patients.
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.
For more information and resources on ILD, please visit the UCSF ILD Program website
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