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Pulmonary Fibrosis

Sidra Saeed, MD, Sara Usman, MBBS and Faisal Saeed, MD Reviewed 06/2019
 


BASICS

DESCRIPTION

  • Pulmonary fibrosis (PF) is a part of interstitial lung diseases (ILDs) that is a family of >200 different lung diseases, which are characterized by inflammation, cellular prolifer...

DIAGNOSIS

HISTORY

  • Very insidious and slow onset

  • Exertional breathlessness and nonproductive cough

  • Constitutional symptoms are uncommon but can present with weight loss, fever, fatigue, myalgia, and arthr...

TREATMENT

GENERAL MEASURES

  • Smoking cessation

  • Pulmonary rehabilitation

  • Referral to a transplant center

  • Supplemental oxygen:

    • Clinical practice guidelines strongly recommend supplemental oxygen.

    • Goal oxyhemogl...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

  • Pulmonary clinic and, if possible, at an ILD clinic.

  • Monitoring with serial HRCT and PFT along with 6MWD and exercise oximetry

  • If on treatment with any of the newly...

REFERENCES

1
Raghu G, Remy-Jardin M, Myers JL, et al; for American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, Latin American Thoracic Society. Diagnosis of idiopathic...

ADDITIONAL READING

CODES

ICD10

  • J84.10 Pulmonary fibrosis, unspecified

  • J84.112 Idiopathic pulmonary fibrosis

ICD9

  • 515 Postinflammatory pulmonary fibrosis

  • 516.31 Idiopathic pulmonary fibrosis

SNOMED

  • 51615001 Fibrosis of lung (di...

CLINICAL PEARLS

  • IPF is most common idiopathic ILD and frequently misdiagnosed and mistreated.

  • HRCT is cornerstone for diagnosis.

  • Pulmonary hypertension is very common.

  • Until recently available two newer ...

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