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
Article

Abstract

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.

Keywords

IPF Pirfenidone N-acetylcysteine Apnea Reflux Transplantation 

Abbreviations

IPF

Idiopathic pulmonary fibrosis

TLC

Total lung capacity

FVC

Forced vital capacity

DLco

Carbon monoxide diffusing capacity

UIP

Usual interstitial pneumonitis

HRCT

High-resolution computed tomographic

FDA

Food and drug administration

CT

Computed tomography

BUILD

Bosentan use in interstitial lung disease

FEV1

Forced expiratory volume in one second

NAC

N-acetylcysteine

GERD

Gastroesophageal reflux disease

OSA

Obstructive sleep apnea

BMI

Body mass index

SLT

Single-lung transplant

BLT

Bilateral-lung transplant

US

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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