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Recent Advances in Chest Medicine |

Therapeutic Approach to Adult Fibrotic Lung Diseases OPEN ACCESS

Ayodeji Adegunsoye, MD; Mary E. Strek, MD
Author and Funding Information

Conflict of Interest: Dr. Strek has received institutional funding for IPF research from Bristol-Myers Squibb, Genentech, Gilead and MedImmune and she serves on a data monitoring committee for Boehringer Ingelheim and participated in an advisory committee for Genentech.

Disclosures: Dr. Adegunsoye is supported by a National Institute of Health grant (T32 HL 07605).

Section of Pulmonary and Critical Medicine, University of Chicago Medicine, 5841 South Maryland MC 6076, Chicago, IL 60637

Correspondence: Deji Adegunsoye, MD, 5841 South Maryland MC 6076 Chicago, IL 60637.


Copyright 2016, . All Rights Reserved.


Chest. 2016. doi:10.1016/j.chest.2016.07.027
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Abstract

Among the interstitial lung diseases (ILD), idiopathic pulmonary fibrosis (IPF), chronic hypersensitivity pneumonitis and fibrotic connective tissue disease related ILD are associated with a worse prognosis with death occurring both from respiratory failure and serious associated co-morbidities. The recent development and approval of the antifibrotic agents nintedanib and pirfenidone, both of which reduced the rate of decline in lung function in patients with IPF in clinical trials, offer hope that it may be possible to alter the increased mortality associated with IPF. Although chronic hypersensitivity pneumonitis and connective tissue disease related-ILD may be associated with an inflammatory component, the evidence for the use of immunosuppressive agents in their treatment is largely limited to retrospective studies. The lack of benefit of immunosuppressive therapy in advanced fibrosis argues for rigorous clinical trials using anti-fibrotic therapies in these types of ILD as well. Patients with fibrotic ILD may benefit from identification and management of associated co-morbid conditions such as pulmonary hypertension, gastroesophageal reflux and obstructive sleep apnea, which may improve the quality of life, and in some cases, survival in affected individuals. Lung transplant evaluation should occur early in patients with IPF and those with other forms of fibrotic ILD as early evaluation may optimize post- transplantation outcomes.


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