Interstitial lung disease (ILD), also known as diffuse parenchymal lung disease (DPLD), refers to a group of lung diseases (including idiopathic pulmonary fibrosis), affecting the 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. Hence the term pulmonary fibrosis has fallen out of favor.
Causes
ILD may be classified according to the cause: -
- Inhaled substances
- Inorganic
- Organic
- Drug induced
- Connective tissue disease
- Infection
- Idiopathic
- Malignancy
- Lymphangitic carcinomatosis
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 prednisolone. Some patients respond to immunosuppressant treatment. Patients with hypoxemia may be given supplemental oxygen.
External links
- University of California, San Francisco Interstitial Lung Disease Center of Excellence
- Coalition for Pulmonary Fibrosis - Research, Education, Support & Hope
- What is Pulmonary Fibrosis?
- AIMIP - Italian Association for IPF - Associazione Italiana
- Dorothy P. and Richard P. Simmons Center for Interstitial Lung Diseases
- University of Chicago Interstitial Lung Disease Program
- PA-IPF - The Pennsylvania Idiopathic Pulmonary Fibrosis State Registry
- University of Cincinnati Interstitial Lung Disease Center
- Huff'n'Puff - Interstitial lung disease support and information site
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Last updated on Monday July 21, 2008 at 13:35:51 PDT (GMT -0700)
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