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Interstitial lung disease
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| Interstitial lung disease Classification and external resources |
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| 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 of the lung:[2] 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.[3] The term is commonly combined with idiopathic in "idiopathic pulmonary fibrosis",[4][5][6] denoting fibrotic ILD that cannot be ascribed to a distinct primary cause.
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[edit] Causes
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ILD may be classified according to the cause.[7] One method of classification is as follows
- Inhaled substances
- Inorganic
- Organic
- Drug induced
- Connective tissue disease
- Infection
- Idiopathic
- Malignancy
- Lymphangitic carcinomatosis
[edit] 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.
[edit] 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,[8] such as prednisolone. Some patients respond to immunosuppressant treatment. Patients with hypoxemia may be given supplemental oxygen.
[edit] References
- ^ 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:. PMID 15879420. http://ajrccm.atsjournals.org/cgi/pmidlookup?view=long&pmid=15879420.
- ^ "Frequently Asked Questions About Interstitial Lung Disease - University of Chicago Medical Center".
- ^ MeSH Pulmonary+fibrosis
- ^ Online 'Mendelian Inheritance in Man' (OMIM) PULMONARY FIBROSIS, IDIOPATHIC -178500
- ^ idiopathic pulmonary fibrosis at Dorland's Medical Dictionary
- ^ Meltzer EB, Noble PW (2008). "Idiopathic pulmonary fibrosis". Orphanet J Rare Dis 3: 8. doi:. PMID 18366757. PMC: 2330030. http://www.ojrd.com/content/3//8.
- ^ Bourke SJ (August 2006). "Interstitial lung disease: progress and problems". Postgrad Med J 82 (970): 494–9. doi:. PMID 16891438.
- ^ "Interstitial lung disease: Treatments and drugs - MayoClinic.com".
[edit] External links
- AIMIP Onlus - Italian Interstitial Lung Diseases No-Profit Organization
- 00736 at CHORUS
- 1476788304 at GPnotebook
- MeSH Pulmonary+Fibrosis
- MedlinePlus Overview pulmonaryfibrosis
- coalitionforpf.org - Coalition for Pulmonary Fibrosis: Pulmonary Fibrosis Patient Services, Education; Funding Research to Find a Cure for PF* Dorothy P. and Richard P. Simmons Center for Interstitial Lung Diseases at University of Pittsburgh Medical Center
- Interstitial Lung Disease Program at University of Chicago
- PA-IPF - The Pennsylvania Idiopathic Pulmonary Fibrosis State Registry at University of Pittsburgh
- Interstitial Lung Disease Center at University of Cincinnati
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