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.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 65-74 |
| Number of pages | 10 |
| Journal | Clinical Reviews in Allergy and Immunology |
| Volume | 44 |
| Issue number | 1 |
| DOIs | |
| State | Published - Feb 2013 |
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Keywords
- Apnea
- IPF
- N-acetylcysteine
- Pirfenidone
- Reflux
- Transplantation
ASJC Scopus subject areas
- Immunology and Allergy
Cite this
Therapeutic update in idiopathic pulmonary fibrosis. / Chan, Andrew; Rafii, Rokhsara; Louie, Samuel; Albertson, Timothy E.
In: Clinical Reviews in Allergy and Immunology, Vol. 44, No. 1, 02.2013, p. 65-74.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Therapeutic update in idiopathic pulmonary fibrosis
AU - Chan, Andrew
AU - Rafii, Rokhsara
AU - Louie, Samuel
AU - Albertson, Timothy E
PY - 2013/2
Y1 - 2013/2
N2 - 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.
AB - 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.
KW - Apnea
KW - IPF
KW - N-acetylcysteine
KW - Pirfenidone
KW - Reflux
KW - Transplantation
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UR - http://www.scopus.com/inward/citedby.url?scp=84872677721&partnerID=8YFLogxK
U2 - 10.1007/s12016-010-8244-9
DO - 10.1007/s12016-010-8244-9
M3 - Article
C2 - 21222174
AN - SCOPUS:84872677721
VL - 44
SP - 65
EP - 74
JO - Clinical Reviews in Allergy and Immunology
JF - Clinical Reviews in Allergy and Immunology
SN - 1080-0549
IS - 1
ER -