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

IPF Checklist

Access this IPF checklist to aid in recognizing IPF

Resources for the Healthcare Professional

Download this Special Report authored by experts in interstitial lung disease for
in‑depth information on IPF, as well as the importance of early recognition and referral

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

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