Interstitial lung disease

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Interstitial lung disease
Classification and external resources
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.

Contents

Causes

The alveoli
The alveoli
Micrograph of usual interstitial pneumonia (UIP). UIP is the most common pattern of idiopathic interstitial pneumonia (a type of interstitial lung disease) and usually represents idiopathic pulmonary fibrosis. H&E stain. Autopsy specimen.
Micrograph of usual interstitial pneumonia (UIP). UIP is the most common pattern of idiopathic interstitial pneumonia (a type of interstitial lung disease) and usually represents idiopathic pulmonary fibrosis. H&E stain. Autopsy specimen.

ILD may be classified according to the cause.[7] One method of classification is as follows:

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

Investigation

Patients with pneumocystis pneumonia can present with interstitial lung disease, as seen in the reticular markings on this AP chest x-ray
Patients with pneumocystis pneumonia can present with interstitial lung disease, as seen in the reticular markings on this AP chest x-ray

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

References

  1. ^ 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. 
  2. ^ "Frequently Asked Questions About Interstitial Lung Disease - University of Chicago Medical Center".
  3. ^ MeSH Pulmonary+fibrosis
  4. ^ "OMIM - PULMONARY FIBROSIS, IDIOPATHIC".
  5. ^ idiopathic pulmonary fibrosis at Dorland's Medical Dictionary
  6. ^ Meltzer EB, Noble PW (2008). "Idiopathic pulmonary fibrosis". Orphanet J Rare Dis 3: 8. doi:10.1186/1750-1172-3-8. PMID 18366757. PMC:2330030. 
  7. ^ Bourke SJ (August 2006). "Interstitial lung disease: progress and problems". Postgrad Med J 82 (970): 494–9. doi:10.1136/pgmj.2006.046417. PMID 16891438. 
  8. ^ "Interstitial lung disease: Treatments and drugs - MayoClinic.com".

External links

v • d • e
Pathology of respiratory system (J, 460-519), respiratory diseases
Upper RT
(including URTIs,
Common cold)
Lower RT/lung disease
(including LRTIs)
Interstitial/
restrictive
External agents/
occupational
lung disease
Other
Obstructive or
restrictive
Pleural cavity/mediastinum
Other/general
see also congenital, neoplasia
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  • This page was last modified on 7 August 2008, at 19:59.

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