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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.

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Fig. 1

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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Acknowledgments

We would like to thank Lisa Pastore and Sandy Algaze for their outstanding editorial contributions to this manuscript.

Financial Disclosure

AC has received speaker honorarium from France Foundation and Intermune (IPF). SL has received speaker honorarium from BI and Astra Zeneca (COPD). TEA has received speaker honorarium from BI and GSK (COPD) and Schering Plough (AECB) and has funded research from Pfizer (hospital-acquired pneumonia).

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Correspondence to Andrew L. Chan.

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Chan, A.L., Rafii, R., Louie, S. et al. Therapeutic Update in Idiopathic Pulmonary Fibrosis. Clinic Rev Allerg Immunol 44, 65–74 (2013). https://doi.org/10.1007/s12016-010-8244-9

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