Interstitial lung disease
|Interstitial lung disease|
|Classification and external resources|
End-stage pulmonary fibrosis of unknown origin, taken from an autopsy in the 1980s.
|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), refers to a group of lung diseases affecting the interstitium (the tissue and space around the air sacs of the lungs).  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.
ILD may be classified according to the cause. One method of classification is as follows: (Mnemonic- Inhaled Drugs Can Infect Idiopathic Malignancy)
- Inhaled substances
- Drug induced
- Connective tissue disease
- Infection (mnemonic- TB is CRAP)
- Lymphangitic carcinomatosis
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.
DLCO 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, such as prednisolone. Some patients respond to immunosuppressant treatment. Patients with hypoxemia may be given supplemental oxygen.
- King TE (August 2005). "Clinical advances in the diagnosis and therapy of the interstitial lung diseases". Am. J. Respir. Crit. Care Med. 172 (3): 268–79. doi:10.1164/rccm.200503-483OE. PMID 15879420.
- "Frequently Asked Questions About Interstitial Lung Disease - University of Chicago Medical Center".
- Bourke SJ (August 2006). "Interstitial lung disease: progress and problems". Postgrad Med J 82 (970): 494–9. doi:10.1136/pgmj.2006.046417. PMC 2585700. PMID 16891438.
- "Interstitial lung disease: Treatments and drugs - MayoClinic.com".
For more information and resources on ILD, please visit the UCSF ILD Program website
- AIMIP Onlus - Italian Interstitial Lung Diseases No-Profit Organization
- 00736 at CHORUS
- 1476788304 at GPnotebook
- Pulmonary Fibrosis at the US National Library of Medicine Medical Subject Headings (MeSH)
- MedlinePlus Overview pulmonaryfibrosis
- coalitionforpf.org - Coalition for Pulmonary Fibrosis: Pulmonary Fibrosis Patient Services, Education; Funding Research to Find a Cure for PF
- GeneReview/NCBI/NIH/UW entry on Pulmonary Fibrosis, Familial
- UCSF Interstitial Lung Disease Program at University of California San Francisco
- Interstitial Lung Disease Program at University of Chicago Medical Center
- Interstitial Lung Disease Center at University of Cincinnati
- PA-IPF - The Pennsylvania Idiopathic Pulmonary Fibrosis State Registry at University of Pittsburgh
- WASOG - World Association for Sarcoidosis and Other Granulomatous Disorders