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.
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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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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DOI: https://doi.org/10.1007/s12016-010-8244-9