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