Lisa H. Lancaster, MD, Marilyn K. Glassberg, MD, and Steven D. Nathan, MD, prepared useful practice aids pertaining to interstitial lung disease management for this CME activity titled "Updates in Interstitial Lung Disease: Making Strides in Accurate Diagnosis and Optimized Treatment." For the full presentation, monograph, complete CME information, and to apply for credit, please visit us at http://bit.ly/2zRd85R. CME credit will be available until July 26, 2019.

1. This Practice Aid has been provided as a quick reference to help learners apply the information to their daily practice and care of patients.
Presented is an algorithm that could be applied when determining the diagnosis in a patient with suspected fibrosing ILD. During stage 1, clinical and radiological data are reviewed in the
context of current guidelines for diagnosis of IPF and other ILDs. When the probability of IPF is felt to be too high after review of clinical and radiological data, the diagnosis is confirmed. If the
probability of IPF is not high, the treating physician considers the utility and feasibility of surgical biopsy to review at stage 2. If a biopsy is obtained, it is reviewed in a multidisciplinary setting to
confirm the final diagnosis of IPF or other ILD.
HRCT: high resolution CT; ILD: interstitial lung disease; IPF: idiopathic pulmonary fibrosis.
1. Based on Salisbury ML et al. Respir Med. 2016;118:88-95.
Two-Stage IPF
Diagnosis Method1
PRACTICE AID
Access the activity,“Updates in Interstitial Lung Disease: Making Strides in
Accurate Diagnosis and Optimized Treatment,”at www.peerview.com/CUH40.
Algorithm for Determining Diagnosis in Patient With Suspected
Fibrosing Interstitial Lung Disease
Stage 1:
Clinical and Radiologic Review
Stage 2:
Histopathology Assessment
Patient presents clinical and/or radiologic evidence of fibrosing ILD
Physician assesses exposures, evidence of connective tissue disease,
physical examination findings
Radiologic data (HRCT) assessed in consultation with thoracic radiologist
Probability of IPF not high Probability of IPF high
• Consider surgical lung biopsy
• Multidisciplinary case review when
biopsy obtained
Alternative ILD diagnosis made IPF diagnosis confirmed
IPF diagnosis confirmed

2. This Practice Aid has been provided as a quick reference to help learners apply the information to their daily practice and care of patients.
IPF: idiopathic pulmonary fibrosis.
1. https://www.mayoclinic.org/diseases-conditions/pulmonary-fibrosis/symptoms-causes/syc-20353690. Accessed May 8, 2018.
2. Ofev (nintedanib) Prescribing Information. http://docs.boehringer-ingelheim.com/Prescribing%20Information/PIs/Ofev/ofev.pdf. Accessed May 8, 2018.
3. Esbriet (pirfenidone) Prescribing Information. https://www.gene.com/download/pdf/esbriet_prescribing.pdf. Accessed May 8, 2018.
Handout for Patients
Diagnosed With IPF
PRACTICE AID
Access the activity,“Updates in Interstitial Lung Disease: Making Strides in
Accurate Diagnosis and Optimized Treatment,”at www.peerview.com/CUH40.
Understanding the Disease1
• Pulmonary fibrosis is a condition in which lung tissue becomes damaged and scarred
• The thickened, stiff lung tissue makes it more difficult for your lungs to work properly, and as pulmonary fibrosis worsens,
you become progressively more short of breath
• Pulmonary fibrosis can be caused by a variety of factors, but often the cause is not known. When a cause can't be found,
the condition is called idiopathic pulmonary fibrosis or IPF
• The lung damage caused by IPF can't be repaired, but therapies can help ease the symptoms, improve quality of life, and slow
decline of lung function
• Medications
• Oxygen therapy
• Pulmonary rehabilitation
• Lung transplantation
• Preventative measures, such as flu and pneumonia vaccines
• The lung damage caused by IPF can't be repaired, but therapies can help ease the symptoms, improve
Treatments Your Doctor May Recommend
Dosage and Administration
Nintedanib2
Pirfenidone3
Medications That May Be Prescribed for IPF
Other Important Strategies to Help You Live With IPF
Notes: ____________________________________________________________________________________________________________
_______________________________________________________________________________________________
_______________________________________________________________________________________________
• Each capsule contains 150 mg of nintedanib
• Take one 150-mg capsule, twice daily
• Take with food
• Take each dose approximately 12 hours apart
• Each capsule contains 267 mg of pirfenidone
• When first starting pirfenidone, your doctor will gradually increase (titrate) the dosage
– Days 1-7: one capsule 3 times daily
– Days 8-14: two capsules 3 times daily
– Days 15 onward: three capsules 3 times daily
• Take with food
• Take each dose at the same time each day
• Remain active and stay in shape
• Eat a healthy diet and maintain a reasonable body weight
• Get plenty of rest
• Stop using tobacco and avoid secondhand smoke
• Learn and practice relaxation techniques
• Join a support group

3. Access the activity,“Updates in Interstitial Lung Disease: Making Strides in
Accurate Diagnosis and Optimized Treatment,”at www.peerview.com/CUH40.
This Practice Aid has been provided as a quick reference to help learners apply the information to their daily practice and care of patients.
1. http://www.pulmonaryfibrosis.org/our-role/community-programs/pff-patient-communication-center. Accessed May 8, 2018.
PFF Patient
Communication Center1
PRACTICE AID
The Patient Communication Center (PCC) responds to inquiries from patients, caregivers,
family members, and healthcare professionals on many topics, including
• Pulmonary fibrosis disease education
• Locating a Pulmonary Fibrosis Foundation (PFF) Care Center or other medical center
• Starting or joining a support group
• Information about clinical trials
• Proactively engaging the pulmonary fibrosis (PF) community
Using a variety of communication channels, the PCC reaches the PF community through
• Dedicated toll-free phone line
• Dedicated email
• Online and in-person support groups
• Social media
• Educational webinars hosted by PF experts
• PFF Ambassadors for programs and events
• Trusted educational resources
As the leading patient advocacy organization for PF, the Pulmonary Fibrosis Foundation offers
a variety of educational resources, including
• Pulmonary Fibrosis Patient Information Guide
• PF Disease Awareness Brochure
• PF Disease Awareness Poster
• Physician Notepad
• Support Group Leader Guide
• Webinars
To speak with someone today, please call 844-TalkPFF (844-825-5733) or email
pcc@pulmonaryfibrosis.org.