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Diffuse Interstitial Lung Disease

Brice T Taylor, MD, J Andrew Woods, BCPS, PharmD and Jacqueline L. Olin, CDCES, BCPS, FASHP, FCCP, MS, PharmD Reviewed 04/2024
 


BASICS

DESCRIPTION

  • Interstitial lung diseases (ILDs) represent a diverse group of chronic progressive lung diseases associated with alveolar inflammation and/or potentially irreversible pulmonary fibro...

DIAGNOSIS

Diagnosis of IPF requires exclusion of other known ILD causes, the presence of a UIP pattern on high-resolution computed tomography (HRCT), and/or surgical lung biopsy pattern  (1). 

HISTORY

  • P...

TREATMENT

  • Evidence does not support the routine use of any specific therapy for ILD in general, especially IPF.

  • No survival benefit of home oxygen use in ILD

  • Corticosteroids have a role in some ILD subt...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Follow-up testing should include PFTs, 6-minute walk test, pulse oximetry, and CXR. 

PATIENT EDUCATION

National Heart, Lung, and Blood Institute: http://www.nhlbi.n...

REFERENCES

1
Raghu G, Martine RJ, Myers JL, et al. Diagnosis of idiopathic pulmonary fibrosis: An official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Resp Crit Care Med. 2018;198:e44-e68.

ADDITIONAL READING

  • Glassberg MK, Minkiewicz J, Toonkel RL, et al. Allogeneic human mesenchymal stem cells in patients with idiopathic pulmonary fibrosis via intravenous delivery (AETHER): a...

CODES

ICD10

  • J84.9 Interstitial pulmonary disease, unspecified

  • J84.10 Pulmonary fibrosis, unspecified

  • J84.111 Idiopathic interstitial pneumonia, not otherwise specified

  • J84.114 Acute interstitial pneumonit...

CLINICAL PEARLS

  • ILD differs from chronic obstructive pulmonary disease (COPD); anatomically, ILD involves the lung parenchyma (i.e., alveoli), and COPD involves both airways and alveoli.

  • In some cases,...

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