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Diagnosis of pulmonary fibrosis

Nurses play a key role in helping patients and their caregivers navigate the diagnostic process1–6

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IRREVERSIBLE LUNG DAMAGE CAUSED BY FIBROTIC ILD UNDERSCORES THE IMPORTANCE OF EARLY DIAGNOSIS7–11

Early and accurate detection of pulmonary fibrosis is essential in ILDs12 as pulmonary fibrosis takes away lung function and once this is lost, it is lost forever.13,14

Identifying pulmonary fibrosis in patients as early as possible may help to improve their burden of disease, slow decline in daily functioning and quality of life, and reduce the risk of early mortality.12,15,16

MULTIDISCIPLINARY EVALUATION IS KEY TO EARLY AND ACCURATE DIAGNOSIS OF FIBROTIC ILDs17,18

Patients should be informed that a diagnosis of ILD requires lung function tests, lung imaging and specialist doctors’ interpretation of their medical history and test results.12,18,19

 

A multidisciplinary team will determine an ILD diagnosis by considering the patient’s medical history and results from PFTs and HRCTs12,18

Clinical assessment

Clinical assessment
PFTs (FVC and DLco)

PFTs (FVC and DLco)
HRCT

HRCT

A multidisciplinary team, including specialist doctors and radiologists, will discuss all available information, which increases the accuracy of ILD diagnosis and prognosis prediction.12,18

DIAGNOSTIC TESTS FOR ILDs

Pulmonary function tests (PFTs) Spirometry and diffusing capacity of the lung for carbon monoxide (DLco)

Pulmonary function tests (PFTs)
Spirometry and diffusing capacity of the lung for carbon monoxide (DLco)
What do they assess? The potential relevance of the test results
Spirometry measures a variety of lung function measures, including forced vital capacity (FVC). FVC is a pulmonary function test that calculates the total amount of air that patients can blow out in one breath
 
DLco measures how efficiently oxygen is transferred from the lungs into the bloodstream20		 Low FVC is usually correlated with worsening lung function and may indicate pulmonary problems, even in asymptomatic patients21

DLco is usually lower in patients with pulmonary fibrosis owing to impaired gas exchange22

  • Pulmonary fuction tests (PFTs), including spirometry and lung diffusion test (DLco), are used to examine lung function12,23,24
  • Prior to testing, patients need to tell the doctor about any medications they are taking, as they may need to stop taking certain medications temporarily (i.e. bronchodilators or other inhaled medications)25,26
  • These tests are quick and straightforward to perform25–27
  • These tests are valuable for observing changes in lung functions over time, so they may be performed regularly12,28

Lung diffusion test (DLco)

  • DLco measures how well the lungs allow oxygen and carbon dioxide to enter and leave the blood20

Spirometry

  • Spirometry is a very low-risk test29 and it measures the rate of air flow and lung size27,30
  • Some patients may feel lightheaded or tired from the required breathing effort30

  • They will be asked to take a deep breath in, before blowing as hard as possible into a tube connected to the spirometer30
  • The doctor may administer medicine to help open the airways to investigate if this changes or improves the test results29,30

  • Clips will be placed on their nose to prevent inhalation through the nostrils
  • A mouthpiece will be placed over their mouth
  • They will be asked to take a breath containing a safe amount of carbon monoxide and hold this for a count of ten
  • They will be asked to rapidly exhale the air held in the lungs, which will be collected and analyzed
HRCT

High-resolution computed tomography (HRCT)

What does it assess? The potential relevance of the test results
Chest HRCT provides detailed images of the lungs and helps to evaluate the extent, distribution and radiological patterns of ILD31–34 

Chest HRCT is the gold standard for early and accurate diagnosis of fibrotic ILDs35		 HRCT is used to characterize the type of ILD and in most cases, helps guide the diagnostic approach36

  • A high-resolution computed tomography scan, also known as an HRCT scan, provides doctors with detailed images of their lungs
  • HRCT lung scans may be used to detect tissue damage and fibrosis2,5,12,23
  • HRCT scans use x-rays, so patients will be exposed to radiation.37 The scanners are designed to make sure patients are not exposed to unnecessarily high levels of radiation.38 Patients should speak to their doctor if they have any concerns38

  • They will be placed on a flat table that moves in and out of a round doughnut‑shaped machine
  • Patients will be positioned so that their chest is in the scanner
  • They will be asked to be as still as possible
  • The doctor/technician will monitor the scan from a separate room
  • The scan will last for approximately 15–30 minutes
Laboratory tests

Laboratory tests39

What do they assess? The potential relevance of the test results
  • Full blood cell count
  • Selected serological tests
  • Urine analysis
 Laboratory tests may help definitively rule out or confirm specific ILDs, including connective tissue disease-associated ILDs39,40

  • Their doctor may carry out a number of additional tests on their blood and urine to make sure that they are diagnosing the correct disease39

A lung biopsy may be needed in some patients. This is a procedure where the doctor takes a small sample of lung tissue to help diagnose ILD.12,40

You have an important role in coordinating diagnostic tests and helping patients understand the practicalities of them2

REGULAR MONITORING FOR PROGRESSION OF ILDs IS VITAL TO INFORM APPROPRIATE CARE41

Pulmonary fibrosis is unpredictable and regular doctor visits can enable detection of progressive disease; patients should expect further testing including lung imaging, lung function tests, as well as monitoring of their symptoms.11,12,30,35,42,43

Patients should be aware that they may be invited to attend PFTs at regular intervals (e.g. every 3–6 months) as this can help to identify progression of ILD.41,44–50

Patients should understand it is important to assess if their ILD is worsening to ensure they receive appropriate care.12,24,42

Diagnosis and monitoring of ILDs12

Diagnosis and monitoring of ILDs

*Standard of care based on specific ILD. †The definition of ILD progession is described in: George PM, et al, Lancet Respir Med. 2020;8:925-934. Adapted from: George PM, et al. Lancet Respir Med. 2020;8:925-954. 
Adapted from: Cottin V, Hirani N, Hotchkin D, et al. Eur Respir Rev. 2018;27(150):180076.

iNSIP AND UNCLASSIFIABLE ILD SHOULD BE A PROVISIONAL DIAGNOSIS AND SHOULD PROMPT FURTHER INVESTIGATION51–54

Unclassifiable ILD and idiopathic nonspecific interstitial pneumonia (iNSIP) are not unique diseases, and so when a patient is provisionally diagnosed, further investigation is required to better understand and characterize the type of ILD.52–54

Regular re-evaluation and the search for new information that might increase the confidence in a specific ILD diagnosis is critical,54 as a specific ILD diagnosis may have implications regarding prognosis and treatment.53

HRCT combined with multidisciplinary discussion can enable a confident ILD diagnosis.17,41,55

You have an important role in recognizing any changes in your patients’ wellbeing that may lead to re-evaluation of their ILD diagnosis2

Case study

Katherine talks about the role of her ILD specialist nurse in her SSc-ILD diagnosis.*

Katherine, age 45, had been experiencing shortness of breath on exertion and a non-productive cough.

Multidisciplinary discussion and review of HRCT scan showed an NSIP pattern and serology was within normal parameters. Failing to identify an underlying cause, the patient was given a diagnosis of iNSIP.

During a routine follow-up appointment with the nurse one year later, Katherine mentioned that she had been recently experiencing pain in the tips of her fingers. The nurse recognized this may be typical of Raynaud’s phenomenon, which is often the earliest clinical manifestation of systemic sclerosis (SSc).56

The nurse reports her findings to the patient’s healthcare team and the rheumatologist requests further serological tests. These results identified serological antibodies that are indicative of SSc-ILD.

This led to a differential diagnosis for Katherine of SSc-ILD.

*Hypothetical patient. Patient case study for illustrative purposes only.

What other resources can help you support your patients in their journey with fibrotic ILD?

Footnotes

  • BAL, bronchoalveolar lavage; DLco, diffusing capacity of the lung for carbon monoxide; FVC, forced vital capacity; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; iNSIP, idiopathic non-specific interstitial pneumonia; MDD, multidisciplinary discussion; NSIP, non-specific interstitial pneumonia; PFT, pulmonary function test; PPF, progressive pulmonary fibrosis; SSc, systemic sclerosis; SSc-ILD, systemic sclerosis-associated interstitial lung disease.

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