Accreditation/Credit Designation

Physicians' Education Resource®, LLC, is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians.

Physicians' Education Resource®, LLC, designates this enduring material for a maximum of 1.5 AMA PRA Category 1 Credits™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Physicians' Education Resource®, LLC, is approved by the California Board of Registered Nursing, Provider #16669, for 1.5 Contact Hours.

Acknowledgment of Commercial Support

This activity is supported by an independent educational grant from Boehringer Ingelheim Pharmaceuticals, Inc.

Medical Crossfire®: Progressive Fibrosis-Interstitial Lung Disease (PF-ILD): Navigating the Complex Pathways to Diagnosis, Management, and Emerging Therapies


Release Date: June 30, 2019
Expiration Date: June 30, 2020
Media: Internet - based

Activity Overview

Interstitial lung diseases (ILDs) are a diffuse group of over 200 lung disorders which are associated with substantial morbidity and mortality. Most of them are rare but when pulmonary fibrosis predominates, they are particularly difficult to treat and manage and patients bear a severely diminished quality of life. Idiopathic pulmonary fibrosis is considered the classic presentation of the fibrotic interstitial lung diseases but other ILD etiologies exhibit similarities with the presence of worsening pulmonary fibrosis and respiratory symptoms, declining lung function, and resistance to immunomodulatory treatments related to other diseases such as rheumatoid arthritis and systemic sclerosis or to environmental exposures and unknown causes.

Early diagnosis and disease intervention are critical for these patients, but lengthy delays and misdiagnoses are common. Typical presenting symptoms are more likely to be an indication of a common respiratory or cardiovascular condition and clinicians will rule out those diagnoses by exposing patients to costly and invasive testing with limited consideration for the possibility of a rare interstitial lung disease. Diagnosis of ILDs is complex and involves careful exclusion of alternative etiologies by a multidisciplinary team integrating findings from clinical, radiologic, and pathologic exam. During this Medical Crossfire®, expert faculty specializing in pulmonology, rheumatology and diagnostic radiology will share their clinical practice insights for making an accurate diagnosis and considering treatment with antifibrotics that can delay disease progression. Faculty will then elaborate on strategies for clinician-patient collaboration to individualize a comprehensive treatment program that addresses comorbidities, patient expectations and best possible quality of life.

Benefits of Participating

  • Gain familiarity with etiologies and early recognizable signs and symptoms of interstitial lung diseases
  • Learn the role of antifibrotics in disease management and how to set patient treatment expectations
  • Understand the benefits of a multidisciplinary approach to diagnosis and ongoing disease management

CME Activity Table of Contents

  • Pre-activity test
  • Module 1: Overview and faculty introductions
  • Module 2: Pathogenetic mechanisms of progressive fibrosing ILDs
  • Module 3: Multidisciplinary diagnosis of fibrosing interstitial lung diseases
  • Module 4: Treatment of PF-ILDs: Considerations, clinical trials, goals and patient expectations
  • Module 5: Clinician-patient collaboration in disease management for an individualized approach
  • Post-activity test
  • Evaluation

Acknowledgement of Commercial Support

This activity is supported by an independent educational grant from Boehringer Ingelheim Pharmaceuticals, Inc.

Instructions for This Activity and Receiving Credit

  • You will need to log in to participate in the activity.
  • Each presentation may contain an interactive question(s). You may move forward through the presentation; however, you may not go back to change answers or review audio files/content until you finish the presentation.
  • At the end of the activity, educational content/audio files will be available for your reference.
  • In order to receive a CME/CE certificate, you must complete the activity.
  • Complete the Posttest and pass with a score of 70% or higher, complete the Evaluation, and then click on “Request for Credit.” You may immediately download a CME/CE certificate upon completion of these steps.


Target Audience

This online educational activity is directed toward pulmonologists, rheumatologists, radiologists, clinical immunologists, allergists, specialty nurse practitioners, physician assistants, primary care clinicians and other healthcare professionals involved in the treatment of patients with PF-ILD.

Learning Objectives

Upon successful completion of this activity, you should be better prepared to:

  • Identify key mechanisms underlying the varied forms of PF-ILD, clinical presentation and management.
  • Assess the pathological patterns of fibrosis in PF-ILD and their role in defining disease etiology and subsequent management.
  • Examine the role of current and emerging agents in the treatment of patients with PF-ILDs.
  • Implement team-based strategies to improve individualization of treatment plans for patients with PF-ILD.

Faculty, Staff, and Planners’ Disclosures

Faculty

Steven Nathan
Steven Nathan, MD
Medical Director, Advanced Lung Disease and Transplant Program
Inova Fairfax Hospital
Professor of Medicine, Virginia Commonwealth University
Inova Fairfax Campus

Disclosures: Grant Research Support: Biogen; Consultant: Roche-Genentech, Boerhinger-Ingelheim, Galapagos, Promedior, Bellerophon, United Therapeutics; Speakers Bureau: Roche-Genentech, Boerhinger-Ingelheim.

Ami N. Rubinowitz
Ami N. Rubinowitz, MD
Associate Professor Thoracic Imaging Section
Department of Radiology and Biomedical Imaging
Yale University School of Medicine, Yale-New Haven Hospital
New Haven, CT

Disclosures: Ami Rubinowitz has no relevant financial relationships with commercial interests to disclose.

Joshua J. Solomon
Joshua J. Solomon, MD
Associate Chief, Section of Critical Care
Associate Professor
Department of Medicine
Division of Pulmonary, Critical Care & Sleep Medicine
National Jewish Health
Denver, CO

Disclosures: Grant Research Support: Pfizer, Boehringer Ingelheim; Speakers Bureau: Genentech.

Monique Hinchcliff
Monique Hinchcliff, MD, MS
Associate Professor of Medicine
Director of Clinical Research
Director of Yale Scleroderma Program
New Haven, Connecticut

Disclosures: Monique Hinchcliff has no relevant financial relationships with commercial interests to disclose.

The staff of Physicians' Education Resource®, LLC (PER®), have no relevant financial relationships with commercial interests to disclose.

Disclosure Policy and Resolution of Conflicts of Interest (COI)

As a sponsor accredited by the ACCME, it is the policy of PER® to ensure fair balance, independence, objectivity, and scientific rigor in all of its CME/CE activities. In compliance with ACCME guidelines, PER® requires everyone who is in a position to control the content of a CME/CE activity to disclose all relevant financial relationships with commercial interests. The ACCME defines relevant financial relationships as financial relationships in any amount occurring within the past 12 months that create a conflict of interest (COI).

Additionally, PER® is required by ACCME to resolve all COI. PER® has identified and resolved all COI prior to the start of this activity by using a multistep process.

Off-Label Disclosure and Disclaimer

This CME/CE activity may or may not discuss investigational, unapproved, or off-label use of drugs. Participants are advised to consult prescribing information for any products discussed. The information provided in this CME/CE activity is for continuing medical education purposes only, and is not meant to substitute for the independent clinical judgment of a physician relative to diagnostic, treatment, or management options for a specific patient’s medical condition.

The opinions expressed in the content are solely those of the individual faculty members, and do not reflect those of PER® or any of the companies that provided commercial support for this program.

PER Pulse Recaps

1 of 3

PER Pulse™ Recap:
Medical Crossfire®: Progressive Fibrosis–Interstitial Lung Disease (PF–ILD): Navigating the Complex Pathways to Diagnosis, Management, and Emerging Therapies

The online continuing medical education activity Medical Crossfire®: Progressive Fibrosis–Interstitial Lung Disease (PF–ILD): Navigating the Complex Pathways to Diagnosis, Management, and Emerging Therapies features a roundtable discussion on the varied forms of PF–ILD and pathological patterns that help define etiology and disease management. Steven Nathan, MD, moderates a discussion among expert faculty Ami N. Rubinowitz, MD; Joshua J. Solomon, MD; and Monique Hinchcliff, MD, MS, to provide insights on early diagnosis of PF–ILDs, treatment options, and strategies for individualization of patient care.

This first of 3 PER Pulse™ Recaps will focus on early recognition of ILD, as explored in Medical Crossfire®: Progressive Fibrosis–Interstitial Lung Disease (PF–ILD): Navigating the Complex Pathways to Diagnosis, Management, and Emerging Therapies.

ILDs are relatively rare and encompass >200 types, which makes diagnosis and disease management clinically challenging. The typical presentation of shortness of breath and possibly cough competes with much more common diseases such as chronic obstructive pulmonary disease or congestive heart failure, leading to misdiagnosis or delays in diagnosis. An early clue to distinguish ILD from other more common conditions is hearing distinctive “crackles” in the lungs. An x-ray may show underlying fibrotic lung disease if there is moderate or more advanced fibrosis, but x-ray is not sensitive enough to visualize early interstitial lung disease. High-resolution CT (HRCT) provides the sensitivity needed for an earlier diagnosis with identification of specific patterns such as usual interstitial pneumonia, which includes subpleural basilar predominant fibrotic changes with stacks of cysts known as honeycombing.1

ILDs can be classified into 5 etiologies to help dictate treatment and define progression:

  • Infectious (cytomegalovirus; influenza)
  • Immunologic (connective tissue disease)
  • Inhalational (chronic hypersensitivity pneumonitis; asbestosis)
  • Iatrogenic (medications)
  • Idiopathic (most common)

Pulmonologists will seek to exclude all other causes before considering idiopathic disease, and identification of underlying connective disease is tantamount. Patients should answer screening questions: Do they have dry eyes or dry mouth, increasing periodontal disease, morning stiffness, swollen fingers, or Reynaud’s syndrome? When an ILD is suspected, the patient’s hands should always be examined.

Key points:

  • Diagnosis and management of PF–ILD is challenging; its early symptoms are similar to those of more common airway diseases
  • HRCT is optimal for diagnosis and visualization of fibrotic disease patterns
  • Patients with suspected ILD should be screened for underlying connective tissue disease

Reference:

  1. Raghu G, Remy-Jardin M, Myers JL, et al; American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society. Diagnosis of idiopathic pulmonary fibrosis. an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2018;198(5):e44-e68. doi: 10.1164/rccm.201807-1255ST.

2 of 3

PER Pulse™ Recap:
Medical Crossfire®: Progressive Fibrosis–Interstitial Lung Disease (PF–ILD): Navigating the Complex Pathways to Diagnosis, Management, and Emerging Therapies

As a follow-up to the online continuing medical education activity Medical Crossfire®: Progressive Fibrosis–Interstitial Lung Disease (PF–ILD): Navigating the Complex Pathways to Diagnosis, Management, and Emerging Therapies, this second of 3 PER Pulse™ Recaps will focus on new and emerging treatment options for PF–ILD.

 

While no cure or treatment guidelines exist PF-ILD, the availability of antifibrotic agents that affect the progression of disease over time has changed the treatment landscape. Nintedanib and pirfenidone, both approved in 2014, demonstrated a reduction in decline of forced vital capacity (FVC) for patients with idiopathic pulmonary fibrosis (IPF) in clinical trials, indicating a slowing of disease progression.1-2. Both agents are being evaluated in ongoing clinical trials for PF–ILD.3 Recently, a phase III trial of nintedanib in systemic sclerosis–associated ILD found that the annual rate of decline in FVC was lower with the agent than with placebo.4

Common adverse effects (AEs) of nintedanib include diarrhea, nausea, and abdominal pain, similar to those of pirfenidone (eg, nausea, rash, abdominal pain).5-6 Joshua Solomon, MD, associate chief, section of critical care, National Jewish Health, Denver, Colorado, commented that discontinuation rates based on these AEs are very low. Patient education helps manage the AEs and improve tolerability.

A debate for pulmonologists is when to initiate treatment for idiopathic pulmonary fibrosis. Many clinicians will start treatment upon diagnosis while others will wait until disease progression occurs. Steven Nathan, MD, medical director of the Advanced Lung Disease and Transplant Program at Inova Fairfax Hospital in Falls Church, Virginia, takes an aggressive approach to prevent permanent loss of lung volume. Even if patients are asymptomatic, he does not consider their disease to be stable.

Key Points:

  • The antifibrotics nintedanib and pirfenidone are indicated for IPF and are being evaluated for other PF–ILDs
  • AEs associated with these 2 antifibrotics are manageable and generally do not lead to discontinuation of treatment
  • Early treatment of PF–ILD can prevent permanent loss of lung volume

References

  1. Richeldi L, du Bois RM, Raghu G, et al; INPULSIS Trial Investigators. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2071-2082. doi: 10.1056/NEJMoa1402584.
  2. King TE Jr, Bradford WZ, Castro-Bernardini S, et al; ASCEND Study Group. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. [published correction appears in N Engl J Med. 2014;371(12):1172]. N Engl J Med. 2014;370(22):2083-2092. doi: 10.1056/NEJMoa1402582.
  3. ClinicalTrials.gov. Identifiers: NCT03820726; NCT02999178; NCT03099187; Accessed August 15, 2019.
  4. Distler O, Highland KB, Gahlemann M, et al; SENSCIS Trial Investigators. Nintedanib for systemic sclerosis-associated interstitial lung disease. N Engl J Med. 2019;380(26):2518-2528. doi: 10.1056/NEJMoa1903076.
  5. Ofev [prescribing information]. Ridgefield, CT: Boehringer Ingelheim Pharmaceuticals, Inc; 2018. www.accessdata.fda.gov/drugsatfda_docs/label/2018/205832s009lbl.pdf. Accessed August 13, 2019.
  6. Esbriet [prescribing information]. South San Francisco, CA: Genentech USA, Inc; 2019. www.gene.com/download/pdf/esbriet_prescribing.pdf. Accessed August 13, 2019.

3 of 3

PER Pulse™ Recap:
Medical Crossfire®: Progressive Fibrosis–Interstitial Lung Disease (PF–ILD): Navigating the Complex Pathways to Diagnosis, Management, and Emerging Therapies

As a follow-up to the online CME activity Medical Crossfire®: Progressive Fibrosis–Interstitial Lung Disease (PF–ILD): Navigating the Complex Pathways to Diagnosis, Management, and Emerging Therapies, this third of 3 PER Pulse™ Recaps will focus on individualization of patient treatment with a multidisciplinary approach.

Patient education and multidisciplinary care are essential for optimizing management of PF–ILD. Receiving a diagnosis of an ILD is life-changing for a patient, and a frank conversation is essential, including discussion of the disease itself, treatment options, and possible progression with potential need for supplemental oxygen. Clinicians should first ask patients what they already believe they know about PF–ILD, to correct any misinformation, including about survival time. Managing patient expectations is also important, because while treatments such as antifibrotics may not actually make them feel better, they do slow disease progression.

Enrollment in clinical trials should be encouraged; help patients with referrals to centers that have access to trials. Centers specializing in ILD may also support lung transplants for the small percentage of patients who may be candidates. According to Steven Nathan, MD, medical director of the Advanced Lung Disease and Transplant Program at Inova Fairfax Hospital in Falls Church, Virginia, it is always easier to evaluate the patient early on for lung transplant rather than wait for them to decompensate and then rush an evaluation to see if they’re a good candidate. Notably, the most common indication for lung transplantation over the last decade has been idiopathic PF–ILD.

Comorbidities that are common with pulmonary fibrosis include gastroesophageal reflux disease, coronary and ischemic heart disease, sleep apnea, and depression, necessitating multidisciplinary care for these patients that includes gastroenterologists, cardiologists, and primary care physicians. Radiologists are integral to the multidisciplinary team and will look for ancillary findings on CT scans beyond the lungs; these include the presence and severity of coronary calcifications, suggestive of pulmonary hypertension, or dilated esophagus, suggestive of a connective tissue disorder such as scleroderma. Once patients develop increased shortness of breath, pulmonary rehabilitation provides numerous benefits.

Key Points:

  • Patient education includes not only disease education but management of treatment expectations
  • Enrollment in clinical trials is empowering to patients
  • Multidisciplinary care is essential to manage common comorbidities seen with PF–ILD

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