Clinical Reviews in Allergy & Immunology

, Volume 44, Issue 1, pp 65–74 | Cite as

Therapeutic Update in Idiopathic Pulmonary Fibrosis

  • Andrew L. Chan
  • Rokhsara Rafii
  • Samuel Louie
  • Timothy E. Albertson


Idiopathic pulmonary fibrosis (IPF) is a disease of the elderly with a mean age at presentation of 66 years. It is the most common type of idiopathic lung fibrosis, and the most lethal, with a median survival of 3 to 5 years after diagnosis. Abnormalities in fibroblast and humoral response mechanisms may play a role in the pathogenesis of fibrosis in IPF. Clinical trials suggest that pirfenidone, an oral antifibrotic agent, N-acetylcysteine, an antioxidant and perhaps anticoagulation, may have some beneficial effect; however, large-scale studies are necessary for confirmation. Immunosuppression with corticosteroids likely does not confer benefit. Lung transplantation has been shown to improve survival in selected IPF patients. Comorbidities accompanying IPF include gastroesophageal reflux, sleep disturbance, pulmonary arterial hypertension, and coronary artery disease amongst others, and ought to be promptly recognized and managed appropriately. While the US Food and Drug Administration has not currently approved any treatments for IPF, patients with IPF should continue to be strongly encouraged to enroll in ongoing clinical trials for this devastating disease.


IPF Pirfenidone N-acetylcysteine Apnea Reflux Transplantation 



Idiopathic pulmonary fibrosis


Total lung capacity


Forced vital capacity


Carbon monoxide diffusing capacity


Usual interstitial pneumonitis


High-resolution computed tomographic


Food and drug administration


Computed tomography


Bosentan use in interstitial lung disease


Forced expiratory volume in one second




Gastroesophageal reflux disease


Obstructive sleep apnea


Body mass index


Single-lung transplant


Bilateral-lung transplant


United States


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Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  • Andrew L. Chan
    • 1
    • 2
  • Rokhsara Rafii
    • 1
    • 2
  • Samuel Louie
    • 1
  • Timothy E. Albertson
    • 1
    • 2
  1. 1.Division of Pulmonary, Critical Care and Sleep MedicineUC Davis School of MedicineSacramentoUSA
  2. 2.VA Northern California Health Care SystemMatherUSA

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