Multidisciplinary Care for IPF
The challenges described above indicate the need for the evaluation of possible ILD by an experienced multidisciplinary team.35 The importance of multidisciplinary care is supported by guidelines and illustrated by studies of diagnostic accuracy. For example, a study from Europe found high 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.36 ATS guidelines specifically recommend collaborative evaluation and management by a multidisciplinary team, including a pulmonologist, a radiologist, and, possibly, a pathologist.7
Ideally, the care team should have experience with diagnosing and managing ILD. For example, radiologists without experience with ILD may not recognize the features of a UIP pattern on HRCT. Indeed, the distinctions between honeycombing (a hallmark feature of the UIP pattern) and traction bronchiectasis or concurrent emphysema and fibrosis can be challenging, even for experienced observers.37 When primary care clinicians recognize features that suggest a possible ILD, they should consider referring the patient to an ILD center, even before an HRCT is performed.
Diagnostic Delays and the Need for a Multidisciplinary Team
A 78-year-old man:
“I had a CT scan [in] 2011, but I felt I had the beginnings of IPF 4 or 5 years prior when I started running and was suddenly wheezing. My primary care doctor of 30 years told me I had bronchitis/athletic asthma, and so I started taking inhalers. I went through a series of misdiagnoses for five years. I was a competitive swimmer doing 10Ks and martial arts, and I finally went in to a pulmonologist because my times for swimming were dropping. I had shortness of breath and weight loss—I dropped 50 pounds the year preceding my diagnosis—but the shortness of breath only bothered me because of my swimming times decreasing. I saw four different pulmonologists in four years trying to confirm my IPF diagnosis.”
Several conditions are frequently comorbid with IPF and may affect patient function, outcomes, and quality of life. A recent retrospective study of 272 patients with IPF found that 12% had no comorbidities, 58% had one to three comorbidities, and 30% had four to seven comorbidities.38 The most common comorbidities were cardiovascular, pulmonary, and oncologic. Predictably, median survival differed significantly by the number of comorbidities, ranging from 66 months among patients with no comorbidities to 48 months among those with one to three comorbidities and 35 months among those with four to seven comorbidities (P=0.004).
Method of Participation
Participants should read the activity information, review the activity in its entirety, and complete the online post-test and evaluation. Upon completing this activity as designed and achieving a passing score on the post-test, you will be directed to a Web page that will allow you to receive your certificate of credit via e-mail or you may print it out at that time.
The online post-test and evaluation can be accessed at:
Inquiries may be directed to Global Academy for Medical Education at
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This activity has been planned and implemented in accordance with the accreditation requirements and policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint providership of Postgraduate Institute for Medicine and Global Academy for Medical Education. The Postgraduate Institute for Medicine is accredited by the ACCME to provide continuing medical education for physicians.
Postgraduate Institute of Medicine designates this enduring material for a maximum of 1.5 AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.
Postgraduate Institute for Medicine is accredited as a provider of continuing nursing education by the American Nurses Credentialing Center’s Commission on Accreditation. This educational activity for 1.5 contact hours is provided by Postgraduate Institute for Medicine.
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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Please technical questions or concerns to Global Academy for Medical Education at 973-290-8225 or email [email protected].
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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29. Rotolo N, Imperatori A, Dominioni L, et al. Efficacy and safety of surgical lung biopsy for interstitial disease. Experience of 161 consecutive patients from a single institution in Italy. Sarcoidosis Vasc Diffuse Lung Dis. 2015;32:251-258.
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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