Not all breathlessness is asthma, COPD, or CHF

SUSPECT PULMONARY FIBROSIS

Your patient who is experiencing chronic breathlessness could have pulmonary fibrosis.

What is Pulmonary Fibrosis?

Pulmonary fibrosis is a common threat across interstitial lung diseases (ILDs), where the buildup of extracellular matrix infiltrating the lung parenchyma leads to fibrosis and causes the lung tissue to become thick and stiff.

Pulmonary fibrosis may continue to progress and cause irreversible damage that compromises function.

There are over 200 types of ILDs. ILDs are rare lung disorders with known and unknown causes.

Pulmonary fibrosis with progression is characterized by:

Lung function
decline
Early mortality
Worsening quality
of life
Worsening respiratory
symptoms

A progressive form of pulmonary fibrosis can develop in ~ 25% of patients with non-idiopathic pulmonary fibrosis (IPF) ILDs, according to a survey of physicians.

References:
Cottin V et al. Eur Respir Rev. 2018;27(150):180076.
Wells AU et al. Eur Respir J. 2018;51(5):1800692.

Below are examples of ILDs that can lead to pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is one of the most common and deadly of the ILDs.

References:
Cottin V et al. Eur Respir Rev. 2018;27(150):180076.
Demedts M et al. Eur Respir J. 2001;18(suppl 32):2s-16s.
Ley B et al. Am J Respir Crit Care Med. 2011;183(4):431-440.
Oldham JM, Noth I. Respir Med. 2014;108(6):819-829.
Ryu JH et al. Mayo Clin Proc. 2007;82(8):976-986.

Recognize and Refer

First-line healthcare professionals play an important role in the early recognition and referral of patients with suspected pulmonary fibrosis to a pulmonary specialist.

Early recognition and referral are critical in ILD since fibrosis, or scarring of the lungs, causes irreversible damage that compromises lung function.

Symptoms can be nonspecific, including cough and dyspnea, and are often mistaken for symptoms of other common diseases such as COPD, bronchitis, emphysema, asthma, and CHF.

References:
Cottin V et al. Eur Respir Rev. 2018;27(150):180076.
Ryu JH et al. Mayo Clin Proc. 2007;82(8):976-986.
Selman M et al. Ann Intern Med. 2001;134(2):136-151.
Zibrak JD, Price D. NPJ Prim Care Respir Med. 2014;24:14054.

References:
Cosgrove GP et al. BMC Pulm Med. 2018;18(1):9.
Schiza S et al. Eur Respir Rev. 2015;24(136):327-339.
Wong C et al. Cureus. 2018;10(10):e3991.
Zibrak JD, Price D. NPJ Prim Care Respir Med. 2014;24:14054.

Misdiagnosis of ILDs is common

Bronchitis, emphysema, asthma, COPD, and CHF are common misdiagnoses.

of patients with
ILD were
misdiagnosed
at least once
Appropriate
diagnosis can
be delayed by
up to 1-2 years
Sometimes
requiring 3 or
more different
doctors

References:
Cosgrove GP et al. BMC Pulm Med. 2018;18(1):9.
Ley B et al. Am J Respir Crit Care Med. 2011;183(4):431-440.
Schoenheit G et al. Chron Respir Dis. 2011;8(4):225-231.
Zibrak JD, Price D. NPJ Prim Care Respir Med. 2014;24:14054.

Misdiagnosis can lead to frequent physician visits, repeated diagnostic tests, exposure to harmful interventions, and ineffective treatment.

Initial tests that can help provide additional clinical clues are:

PFTs: restrictive
pattern, reduced
FVC and DLCO
Exercise tests:
O₂ desaturation
with exercise
Chest X-ray: lung
abnormalities; however,
normal lungs on radiograph
does not rule out ILDs HRCT: provides more accurate
picture of the lungs and reveals
common features of ILD

References:
Cosgrove GP et al. BMC Pulm Med. 2018;18(1):9.
Ryu JH et al. Mayo Clin Proc. 2007;82(8):976-986.
Zibrak JD, Price D. NPJ Prim Care Respir Med. 2014;24:14054.

If you suspect a patient with pulmonary fibrosis, refer them to your local pulmonologist or interstitial lung disease (ILD) center for a complete evaluation.

RECOGNIZE SYMPTOMS EARLY

Chronic nonproductive cough
Dyspnea on exertion
Velcro^®-like crackles on lung auscultation

READ TESTS AND SCANS FOR EVIDENCE OF ILD

Restrictive pattern on PFTs
Reduced FVC and DLCO
Abnormalities on chest X-ray
- HRCT scans are needed for a more accurate picture of the lungs

REFER TO A LUNG SPECIALIST IF ILD IS SUSPECTED

Once you suspect ILD, refer patients to a lung specialist who can provide specialized expertise and further analysis of HRCT scans

References:
Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824.
Ryu JH et al. Mayo Clin Proc. 2007;82(8):976-986.
Wong C et al. Cureus. 2018;10(10):e3991.
Zibrak JD, Price D. NPJ Prim Care Respir Med. 2014;24:14054.

Accurate and early diagnosis is crucial to ensure that patients receive timely intervention to allow for disease management.

Reference:
Cottin V et al. Eur Respir Rev. 2018;27(150):180076.

IPF is one of the most common and deadly of the ILDs
Access the following tools to aid in your recognition and referral

Diagnosing IPF

This video will provide a brief overview to aid a healthcare professional to recognize a potential patient with IPF, encouraging a referral to a pulmonologist if IPF is suspected

Resources for the Healthcare Professional

Download this flashcard for helpful tools on how to C.H.E.C.K. for ILD in your patients.

Download and Listen Now! This one sound can help diagnose a fatal lung disease

Download Developed in partnership with the American College of Chest Physicians (CHEST) through its Interstitial and Diffuse Lung Disease NetWork, this questionnaire assists HCPs in performing a comprehensive patient medical history.

Download this Special Report written by Dr. Jonathan Ilowite, of UBM Medica, which provides guidance on the need for healthcare professionals to be aware of IPF, in an effort to encourage faster referrals to pulmonologists

Watch Dr. Michael Zlupko as he provides an overview of common ILDs and reviews the signs and symptoms of disease progression. Dr. Zlupko will also highlight the significance of comprehensive care for patients with ILD.

Test Your Knowledge:

Further Information

Mobilizes people and resources to provide access to high‑quality care

Pulmonary fibrosis resources, education, and support options for healthcare professionals

IPF education and resources for healthcare professionals

IPF awareness and advocacy

Once an IPF diagnosis is confirmed, this site is for patients and caregivers

Healthcare professional education and resources for pulmonary involvement in patients with systemic sclerosis

Educational resource regarding rheumatoid arthritis and
lung disease

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