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For Canadian healthcare professionals only

ILDs can be progressive and unpredictable1

Acute exacerbations can be fatal and strike at any time in pulmonary fibrosis1

Progressive pulmonary fibrosis is associated with decreased quality of life2

Early and regular monitoring are important to detecting progression and can help inform management decisions and patient counselling3,4

According to an online survey, physicians estimated that patients have a survival time of
2.5–4 years after detection of progressive fibrosis4 

In IPF, lung function declines regardless of the clinical course of disease progression5

Graph showing disease progression in IPF. Click graph full text for description.

Adapted from Ley, et al.

Graph full text

In IPF, baseline FVC doesn’t predict disease progression6,7 

FVC has already declined before diagnosis7

Predicted FVC can appear to be greater than 100% at baseline7

Emphysema artificially increases FVC6,7

In IPF, FVC reduction is
directly linked to physical
activity reduction8*

ca
Equation showing how a 1% reduction in predicted FVC is equated with 60 fewer steps taken per day.

Risk factors for progression in HP9

  • Older age

  • Smoking history

  • Lower baseline FVC % predicted

  • Lack of identification of an inciting antigen

Risk factors for progression in iNSIP10

  • Male sex

  • Initial TLC

  • Decline in FVC at 3 months

  • Presence of honeycombing on HRCT

Risk factors for progression in RA-ILD11–13

  • Extent of ILAs on HRCT

  • UIP pattern on HRCT

  • Lower baseline FVC or DLco

  • High baseline concentration
    of KL-6

Risk factors for progression in SSc-ILD14–18

  • Male sex

  • Older age

  • dcSSc subtype

  • High baseline mRSS

  • Elevated KL-6 level

What should you do for patients with connective tissue disease ILD?19–22

Make regular PFTs routine in patients susceptible to connective tissue disease ILD

  • Conduct proactive and regular monitoring for any deterioration in respiratory symptoms such as cough or difficulty breathing
  • For at-risk patients, HRCT should be evaluated at the first suspicion of ILD involvement, if possible at baseline diagnosis, and repeated upon worsening of either PFT or respiratory symptoms

Regardless of ILD diagnosis, older patients with a UIP pattern of pulmonary fibrosis on HRCT decline at the greatest rate23

Early and regular monitoring can help detect a progressive fibrosing disease course21

Monitoring for ILD progression

PFTs6,23–25

  • Deterioration in PFTs should trigger urgency for further investigation

  • DLco is among the first parameters to decline in progressive ILDs

  • DLco decline is especially indicative of progression when accompanied by a decline in FVC or increased radiological evidence of fibrosis on HRCT over time

HRCT26–29

  • Evaluate HRCT upon worsening of PFT scores or respiratory symptoms

Other indicators for progressive fibrosis23

  • Acute exacerbation

  • Decline in the 6MWD

Learn more about early screening and diagnosis

Time is of the essence when progressive fibrosing ILD is suspected 

Refer patients with a progressive fibrosing ILD for pulmonary rehabilitation as soon as possible to help keep them active and preserve their quality of life.5

ILD=interstitial lung disease; IPF=idiopathic pulmonary fibrosis; FVC=forced vital capacity; QoL=quality of life; HP=hypersensitivity pneumonitis; iNSIP=idiopathic nonspecific interstitial pneumonia; TLC=total lung capacity; HRCT=high-resolution computed tomography; CTD-ILD=connective tissue disease-associated interstitial lung disease; RA-ILD=rheumatoid arthritis-associated interstitial lung disease; ILA=interstitial lung abnormality; DLco=diffusing capacity of the lungs for carbon monoxide; dcSSc=diffuse cutaneous systemic sclerosis; mRSS=modified Rodnan Skin Score; UIP=usual interstitial pneumonia; PFT=pulmonary function test; 6MWD=6-minute walking distance.

  • *

    Study of 48 patients with IPF. Average steps per day: 5,017±3,360. Steps per day measured for one week, 24 hours per day, by use of the SenseWear Armband (Body, 499AMedia Inc., Pittsburgh PA, USA), an activity monitor that has been widely used in patients with IPF and chronic obstructive pulmonary disease.8

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