What is IPF?
IPF is the most common and lethal of the idiopathic interstitial pneumonias, a subgroup of the family of interstitial lung diseases (ILDs) (Figure 1).12 ILDs involve inflammation of the interstitium, or the tissue and spaces around the alveoli of the lungs. IPF accounts for about 20% of all ILDs and is the most common and severe of the idiopathic interstitial pneumonias.12
IPF is clinically characterized by nonspecific symptoms, such as dyspnea on exertion and dry cough, and requires specific investigations (eg, high-resolution computed tomography [HRCT]) for diagnosis. The disease is associated with aging (median age of onset, 65-70 years) and is more common in men and patients with a history of smoking.7
Features of IPF that cloud understanding of the disease include its variable natural history, a high rate of complicating comorbidities, a paucity of accurate indicators of disease progression, and limited insight into its pathogenesis.13,14 Other factors contributing to suboptimal management include variation among providers in the application of guideline recommendations and in the analysis of histologic and radiographic studies.14
The pathophysiology of IPF is poorly understood but likely involves multiple pathways and complex interactions between genetic, epigenetic, metabolic, and environmental factors.15 Indeed, the diversity of presentations, findings, and courses of disease in IPF suggests that fibrosis results from multiple pathogenic pathways, each of which may be influenced by endogenous and environmental factors.16 This multifactorial pathogenesis may explain the often poor results from clinical trials of agents that target individual mediators or pathways in IPF.15
The current model for the pathogenesis of IPF involves abnormal wound healing in response to persistent or recurrent injuries by environmental factors (eg, cigarette smoke, microaspirations, infection, dusts) on a background of genetic susceptibility.15,17 The result is an imbalance between profibrotic and antifibrotic mediators, leading to excessive deposition of extracellular matrix components in the interstitium and alveolar spaces of the lungs. Progressive interstitial fibrosis and scarring destroy the lung microarchitecture and compromise lung function.15 As a result, gas exchange is impaired. Although oxygen tension may be normal or near normal in IPF, diffusing capacity of the lungs for carbon monoxide (DLCO) is often reduced.
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The identification and management of patients with idiopathic pulmonary fibrosis (IPF) is replete with challenges. The symptoms of IPF are common to a variety of pulmonary conditions and easily missed by patients and clinicians. Suspicion of IPF often does not arise for at least several months after the onset of symptoms. Indeed, a key and common reason contributing to delayed diagnosis is the lack of expertise in IPF among community physicians. Primary care clinicians and pulmonary therapists may be unaware of the core signs, symptoms, and tests for IPF and its differential diagnosis. The early symptoms of IPF, dyspnea on exertion and dry cough, are often ignored by patients and primary care physicians and are attributed to aging or smoking. Because IPF most commonly occurs in men, older individuals (>50 years), and those with a history of smoking, clinicians may be focused on more common causes of pulmonary symptoms, especially in the community setting.
Studies of IPF also found that a multidisciplinary approach, including radiologists, pathologists, and pulmonologists, improved diagnostic agreement at both academic and community sites. A study from Europe found good accuracy for the diagnosis of IPF at expert centers (approaching 90%), but the level of agreement within the expert teams was only fair to moderate. Together, these findings suggest that a multidisciplinary team is essential, even in tertiary care settings. Indeed, the American Thoracic Society (ATS) guidelines specifically recommend a multidisciplinary approach to diagnosis and management.
The availability of new therapies shown to slow disease progression highlights the need for earlier diagnosis and intervention in IPF. It has been suggested that a "window of opportunity" may exist during which treatment can promote optimal outcomes. Furthermore, delays in diagnosis may limit treatment options, as well as increase costs, reduce patient quality of life, and impact survival.
At the conclusion of this program, participants should be better able to:
- Utilize the signs, symptoms, and epidemiology of IPF to better recognize patients in need of specialty care
- Employ a multidisciplinary care team approach for IPF to enhance patient outcomes
- Provide timely referral of patients with IPF to tertiary care to improve survival
- Review clinical trial data supporting the efficacy and safety of pirfenidone and nintedanib
- Select appropriate pharmacologic therapy for patients with IPF
- Communicate with patients with IPF and provide effective disease state education
Postgraduate Institute for Medicine (PIM) requires instructors, planners, managers, and other individuals who are in a position to control the content of this activity to disclose any real or apparent conflict of interest (COI) they may have as related to the content of this activity. All identified COI are thoroughly vetted and resolved according to PIM policy. PIM is committed to providing its learners with high-quality CME/CE activities and related materials that promote improvements or quality in healthcare and not a specific proprietary business interest of a commercial interest.
The faculty reported the following financial relationships or relationships to products or devices they or their spouse/life partner have with commercial interests related to the content of this CME/CE activity:
Gregory P. Cosgrove, MD, FCCP: Dr. Cosgrove has been a consultant for: Boehringer Ingelheim, Genentech, Global Blood Therapeutics and Veracyte.
Jonathan H. Chung, MD: Dr. Chung has no relevant financial relationships to disclose.
Kristin L. DeSimone, MD: Dr. DeSimone has no relevant financial relationships to disclose.
Global Academy for Medical Education Staff: Sylvia H. Reitman, MBA, DipEd; Mike LoPresti; Shirley V. Jones, MBA; Ron Schaumburg; and Josh Kilbridge, hereby state that they or their spouse/life partner do not have any financial relationships or relationships to products or devices with any commercial interest related to the content of this activity of any amount during the past 12 months.
Postgraduate Institute for Medicine: The following planners and managers, Trace Hutchison, PharmD; Samantha Mattiucci, PharmD, CHCP; Judi Smelker-Mitchek, MBA, MSN, RN; and Jan Schultz, MSN, RN, CHCP, have nothing to disclose.
Off-Label/Investigational Use Disclosure
This educational activity may contain discussion of published and/or investigational uses of agents that are not indicated by the US Food and Drug Administration. The planners of this activity do not recommend the use of any agent outside of the labeled indications.
This activity is not an official program of the American College of Chest Physicians (CHEST) and accordingly is not accredited by CHEST®.
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Copyright © 2016 by Global Academy for Medical Education, LLC, Frontline Medical Communications Inc., and its Licensors. All rights reserved. No part of this publication may be reproduced or transmitted in any form, by any means, without prior written permission of the Publisher. Global Academy for Medical Education, LLC, will not assume responsibility for damages, loss, or claims of any kind arising from or related to the information contained in this publication, including any claims related to the products, drugs, or services mentioned herein.
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This continuing education supplement was developed from interviews with the faculty. It is the final activity in the three-part curriculum, Improving the Diagnosis and Management of IPF Through Simulation and Peer Benchmaring. The supplement content brings together the key teaching points from the previous two online simulations, which may be found at: http://www.globalacademycme.com/ supplements/clinical-decision-makingin- ipf-management.html.
The faculty acknowledge the editorial assistance of Global Academy for Medical Education, LLC, and Josh Kilbridge, medical writer, in the development of this supplement.
Neither the editors of CHEST Physician nor the Editorial Advisory Board nor the reporting staff contributed to its content. The ideas and opinions expressed are those of the faculty and do not necessarily reflect the views of the supporters, Global Academy for Medical Education, Postgraduate Institute for Medicine, or the Publisher. Please refer to the official prescribing information for each product for discussion of approved indications, contraindications, and warnings.
Participants have an implied responsibility to use the newly acquired information to enhance patient outcomes and their own professional development. The information presented in this activity is not meant to serve as a guideline for patient management. Any procedures, medications, or other courses of diagnosis or treatment discussed or suggested in this activity should not be used by clinicians without evaluation of their patient’s conditions and possible contraindications and/or dangers in use, review of any applicable manufacturer’s product information, and comparison with recommendations of other authorities.
Copyright © 2016 by Global Academy for Medical Education, LLC, Frontline Medical Communications Inc., and its Licensors. All rights reserved. No part of this publication may be reproduced or transmitted in any form, by any means, without prior written permission of the Publisher. Global Academy for Medical Education, LLC, will not assume responsibility for damages, loss, or claims of any kind arising from or related to the information contained in this publication, including any claims related to the products, drugs, or services mentioned herein