Diffuse Interstitial Lung Disease

Reviewed 06/2017
 


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

  • Accurate diagnosis is imperative, as treatment choices and prognosis can vary with pathogenesis.

  • Diagnosis of IPF requires exclusion of other known ILD causes, the presence of a UIP pattern o...

TREATMENT

  • Evidence does not support the routine use of any specific therapy for ILD in general, and especially IPF 6,8.

  • No survival benefit of home oxygen use in ILD 9[A].

  • Corticosteroids have a role in...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Follow-up testing should include PFTs, cardiopulmonary stress test, pulse oximetry, and CXR. 

PATIENT EDUCATION

National Heart, Lung, and Blood Institute: http://ww...

REFERENCES

Travis  WD, Costabel  U, Hansell  DM, et al. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of t...

ADDITIONAL READING

  • Martinez FJ, de Andrade JA, Anstrom KJ, et al. Randomized trial of acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med.  2014;370(22):2093–2101.

  • Noth I, Anstrom KJ, ...

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); ILD involves the lung parenchyma (i.e., alveoli) and COPD involves both airways and alveoli.

  • In some cases, avoiding or mi...

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