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Impact of CTD-ILDs

Interstitial lung disease (ILD) is a key driver of early mortality in connective tissue diseases (CTDs)1–6

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FIBROTIC CTD-ILDs ARE CHARACTERIZED BY PULMONARY FIBROSIS THAT CARRIES THE THREAT OF EARLY MORTALITY1–5

5-year mortality rates of CTD-ILDs7–13

Mortality rate estimates for different CTD-ILDs vary between different studies. 5-year mortality rates shown below are based on a range of different estimates from different studies7–13

5-year mortality rate of CTD-ILDs with RA-ILD having the highest rate

 

5-year mortality rates sourced as midpoint values from ranges for CTD-ILD 5-year mortality rate as follows: RA-ILD 5-year mortality rate 35%-39%;7,8 SSc-ILD 5-year mortality rate 10%-18%;9 primary Sjögren’s syndrome-ILD
5-year mortality rate 12%-16%;10–12 PM/DM/CADM (polymyositis/dermatomyositis/clinically amyopathic dermatomyositis) 35% 5-year mortality rate.13

SSC-ILD Mortality

ILD is the leading cause of death in SSc14     

Learn more about the impact on mortality in SSc-ILD
RA-ILD Mort

ILD is an important cause of mortality in RA15,16 

Learn more about the impact on mortality in RA-ILD
pSS-ILD Mortality

ILD increases the risk of mortality in primary Sjögren’s syndrome10–12,17

Learn more about the impact on mortality in primary Sjögren’s syndrome-ILD

REMEMBER THE RISK FACTORS ASSOCIATED WITH EARLY MORTALITY WHEN ASSESSING YOUR PATIENTS WITH CTD-ILDs

Risk factors for early mortality in CTD-ILDs

HRCT
  • UIP pattern18

  • Traction bronchiectasis19

  • Honeycombing19,20

Lung function
  • Lower baseline DLCO18

  • Reduction in DLCO19

  • Lower baseline 6MWD18

Demographics
  • Male sex18

  • Older age18

Current approaches to lung function monitoring in CTD-ILDs have been influenced by IPF studies, in which serial FVC and DLCO decline has been predictive of earlier mortality21

In SSc, DLCO <40%, FVC <60%, and a restrictive FEV1/FVC ratio are predictive of a marked increase in mortality21

EXTENT OF HONEYCOMBING ON HRCT AS WELL AS DLCO ARE STRONGLY ASSOCIATED WITH MORTALITY IN FIBROTIC CTD-ILDs19,22

Honeycombing present on HRCT is associated with an increased long-term mortality rate in patients with CTD-ILDs vs. no honeycombing present (p<0.001)20
Increased long-term mortality rate in patients with CTD-ILDs with honeycombing on HRCT compared with no honeycombing

10-year survival in patients with CTD-ILDs with honeycombing present or absent.
Reprinted from Annals of the American Thoracic Society, 16(5), Adegunsoye et al, Computed Tomography Honeycombing Identifies a Progressive Fibrotic Phenotype with Increased Mortality across Diverse Interstitial Lung Diseases, 580-588, ©2022.

Find out how to investigate the radiographic characteristics of CTD-ILDs with HRCT

Lower baseline DLCO and reduction in DLCO are associated with increased mortality in fibrotic CTD-ILDs18,19
Lower baseline DLco and reduction in DLco are associated with increased mortality in fibrotic CTD-ILDs
Learn about the risk factors for early mortality in SSc-ILD
Learn about acute exacerbation of ILD in RA-ILD
Learn about acute exacerbation of ILD in primary Sjögren’s syndrome-ILD

ALTHOUGH RARE, ACUTE EXACERBATION OF ILD IS A DEADLY THREAT IN CTD-ILDs THAT COULD STRIKE AT ANY TIME23-27

Based on patients with IPF, acute exacerbation of ILD is most likely triggered by an acute event, such as infection28
Acute exacerbation

Acute exacerbation of ILD is characterized by rapid respiratory deterioration with severe hypoxemia22,26,27,29,30  
 

Learn about acute exacerbation of ILD in SSc-ILD

 
ACUT

Acute exacerbation of ILD can occur in patients with CTD-ILDs at any point during the course of disease22,26,27,29,30  
 

Learn about acute exacerbation of ILD in RA-ILD

 
Post exacerbation

Post-exacerbation hospital mortality in patients with CTD-ILDs is reported to range from 50%–100%23 
 

Learn about acute exacerbation of ILD in primary Sjögren’s syndrome-ILD

 

COUGH AND ACTIVITY-LIMITING DYSPNEA HAVE AN IMPACT ON THE EVERYDAY LIVES OF PATIENTS WITH CTD-ILDs31–33

Symptoms of CTD-ILDs are generally non-specific, such as exertional dyspnea, cough, or fatigue3,4
Cough

Cough

Dyspnea

Dyspnea

Fatigue

Fatigue

Cough and dyspnea have been found to be associated with frustration, shame, anger and isolation among patients, leading to a loss in participation in pleasurable activities, such as walking, dancing or playing with children31

* ILD-related cough adversely impacts physical function, social participation, activities of daily living, and sleep quality31

* Dyspnea impacts the patients’ ability to perform functional activities and life priorities31

CTD-ILD has been associated with both short-term daily disability and long-term difficulties with life planning31

* Patients with CTD-ILDs live with uncertainty and anxiety, and can struggle over self-identity31

* Patients with progressive CTD-ILDs suffer from emotional distress that may include feelings of depression, anxiety and loss of independence34,35

 
How can you identify fibrotic ILD early in your patients with CTDs?

 

Footnotes

6MWD, six-minute walking distance; CADM, clinically amyopathic dermatomyositis; CTD, connective tissue disease; CTD-ILD, connective tissue disease-associated interstitial lung disease; DLCO, diffusing capacity of the lung for carbon monoxide; DM, dermatomyositis; FVC, forced vital capacity; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; PM, polymyositis; RA, rheumatoid arthritis; rheumatoid arthritis-associated interstitial lung disease; SSc, systemic sclerosis; SSc-ILD, systemic sclerosis-associated interstitial lung disease.

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  20. Adegunsoye A, Oldham JM, Bellam SK, et al. Computed Tomography Honeycombing Identifies a Progressive Fibrotic Phenotype with Increased Mortality across Diverse Interstitial Lung Diseases. Ann Am Thorac Soc. 2019;16:580–588.

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