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Pulmonary Fibrosis

Reviewed 06/2018
 


BASICS

DESCRIPTION

  • Characterized by fibrosis of the lung parenchyma

  • Chest CT shows reticular pattern and honeycombing, with subpleural and lower lobe predominance.

  • Lung biopsy shows “usual interstitial p...

DIAGNOSIS

HISTORY

  • Slowly progressive dyspnea

  • Dry nonproductive cough

PHYSICAL EXAM

  • Tachypnea

  • Fine inspiratory bibasilar crackles

  • Possible clubbing

DIFFERENTIAL DIAGNOSIS

  • Asbestosis

  • Berylliosis

  • Coal worker pneum...

TREATMENT

GENERAL MEASURES

  • Pulmonary rehabilitation (1)[C]

  • Consider treating for GERD, even if asymptomatic (1)[B].

  • Consider lung transplant evaluation.

MEDICATION

First Line

  • Supplemental oxygen, if needed ...

ONGOING CARE

FOLLOW UP RECOMMENDATIONS

Patient should follow-up with a pulmonologist. 

Patient Monitoring

Disease progression can be monitored by periodic PFTs and HRCT. 

DIET

No specific dietary requireme...

REFERENCES

1
Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Me...

ADDITIONAL READING

  • Baddini-Martinez J, Baldi BG, Costa CH, et al. Update on diagnosis and treatment of idiopathic pulmonary fibrosis. J Bras Pneumol.  2015;41(5):454–466. [View Abstract on OvidM...

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

  • Pulmonary fibrosis can usually be diagnosed based on characteristic chest CT findings, which include a reticular pattern, traction bronchiectasis, and honeycombing with a peripheral an...

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