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Pulmonary fibrosis
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Pulmonary fibrosis

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Contributors: David Sullo MD
Other Resources UpToDate PubMed

Synopsis

Pulmonary fibrosis is a chronic progressive irreversible fibroproliferative (nonneoplastic) disease of the lungs and the most common cause of interstitial pneumonia. The condition is characterized by the formation of scar tissue in the lungs. Pulmonary fibrosis may occur secondary to other disorders, or it may be idiopathic (most common). Typical signs and symptoms include exertional dyspnea, chronic dry cough, crackles, and fatigue. Clubbing occurs in about half of patients and, in the later stages of illness, right heart failure and cor pulmonale may be noted. Onset is typically age 50-70, with smokers at higher risk. It may be associated with pharmaceuticals, radiation therapy, and environmental exposures.

Prognosis is poor for severely progressed cases due to impaired breathing, concomitant illness, and complications. Life expectancy may be only 3-5 years following diagnosis.

Without a known cure, management focuses on slowing progression, prevention of complications, supplemental oxygen, and medications and treatment therapies to ease symptoms. Lung transplant may be considered.

Codes

ICD10CM:
J84.10 – Pulmonary fibrosis, unspecified

SNOMEDCT:
51615001 – Fibrosis of lung

Best Tests

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Updated: 03/15/2018
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Pulmonary fibrosis
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Pulmonary fibrosis : Fatigue, Exertional dyspnea, Dyspnea, Crackles, Dry cough
Copyright © 2019 VisualDx®. All rights reserved.