Mortality of ILDs

Pulmonary fibrosis is a key driver of early mortality in patients with fibrotic ILDs1–8

ILD patients with the HRCT scans in their hands.

ILDs characterized by pulmonary fibrosis carry the threat of early mortality9–12

5-year mortality rates of non-IPF ILDs7,13–19

5-year mortality rate of non-IPF ILDs

5-year mortality rate for iNSIP sourced from a retrospective study.7 5-year mortality rates for unclassifiable ILD, RA-ILD, SSc-ILD and pSS-ILD sourced as midpoint values from ranges for 5-year mortality rates as follows: unclassifiable ILD 5-year mortality rate 30%–54%.13 RA-ILD 5-year mortality rate 35%–39%.14,15 SSc-ILD 5-year mortality rate 10%–18%.16 pSS-ILD 5-year mortality rate 12%–16%.17–19

PATIENTS WITH NON-IPF ILDs MAY DEVELOP A PROGRESSIVE FIBROSING PHENOTYPE, WHICH CARRIES THE RISK OF EARLY MORTALITY, LIKE IPF20

The progressive fibrosing phenotype is characterized by increased extent of fibrosis, worsening respiratory symptoms and lung function decline9,21–23

Patients diagnosed with IPF, or a non-IPF ILD with progressive pulmonary fibrosis, face a dismal prognosis20,24

Patients diagnosed with IPF have a median survival of 2–5 years24,25

HOW DOES THE PROGNOSIS OF IPF COMPARE TO OTHER DISEASES?

IPF has a worse 5-year survival than many cancers, including breast, prostate, colon, skin cancer, and lymphoma8

Lower 5-year survival rate for IPF compared with many common cancers

Reproduced with permission of the © ERS 2021. European Respiratory Journal 35 (3) 496-504; DOI: 10.1183/09031936.00077309 Published 28 February 2010.

Patients with ILDs, such as RA-ILD, have a high risk of mortality,9–11 but the threat is highest in patients with IPF26

Lower cumulative survival for IPF compared with different HRCT patterns of RA-ILD

Kaplan-Meier curve demonstrating mortality for IPF patients vs. RA-ILD patients subdivided according to Fleischner Society IPF diagnostic guidelines on HRCT pattern.
Reproduced with permission of the © ERS 2021. European Respiratory Journal 53 (1) 1800869; DOI: 10.1183/13993003.00869-2018 Published 3 January 2019.
RA-ILD definite UIP pattern (honeycombing): 48% 3-year and 45% 6-year survival, n=55. Mean survival 3.5 years
RA-ILD probable UIP pattern (no honeycombing): 76% 3-year and 58% 6-year survival, n=56. Mean survival 4.6 years
RA-ILD non-UIP pattern: 92% 3-year and 75% 6-year survival, n=46. Mean survival 5.3 years
IPF: 42% 3-year and 18% 6-year survival, n=284. Mean survival 2.9 years

Compared with fibrotic NSIP, patients with IPF have a worse survival rate27

Lower survival of IPF compared with fibrotic NSIP

Reprinted from Annals of the American Thoracic Society, 171(6), Jegal et al, Physiology Is a Stronger Predictor of Survival than Pathology in Fibrotic Interstitial Pneumonia, 639-644, ©2022.

The 5-year survival rate of patients with fibrotic NSIP (n=41) was 76.2% vs. 43.8% of patients with IPF (n=131) (p=0.007).

6-month changes in FVC and initial DLco, and male sex, are statistically significant predictors of survival in patients with fibrotic NSIP and IPF27

 

Hazard Ratio

95% CI

P Value

Age

1.027

0.992–1.064

0.134

Sex*

2.724

1.277–5.813

0.010

NSIP diagnosis

0.854

0.349–2.093

0.730

Initial FVC, % predicted*

0.987

0.964–1.010

0.262

Initial DLco, % predicted*

0.972

0.949–0.996

0.022

6-month change in FVC*

0.925

0.893–0.958

<0.001

Resting PaO2

0.995

0.961–1.031

0.798

Results of multivariate analysis of prognostic factors of patients with fibrotic NSIP (n=41) and IPF (n=131) after 6 months of follow-up.
*  Analyzed as continuous variables.
Reprinted from Annals of the American Thoracic Society, 171(6), Jegal et al, Physiology Is a Stronger Predictor of Survival than Pathology in Fibrotic Interstitial Pneumonia, 639-644, ©2022.

PATIENTS WITH FIBROTIC ILDs ARE AT RISK OF EARLY MORTALITY20

Icon representing estimated 2,5-4 years survival time for non-IPF progressive fibrosing ILDs.

Patients with non-IPF progressive fibrosing ILD have an estimated survival time of 2.5–4 years20*†

Icon showing that 10-15% patient with RA-ILD are at risk of early mortality.

In patents with RA, ILD triples the risk of death28 and accounts for 10%–20% of deaths29–31

Icon showing that ILD causes around 35% of SSc-related deaths.

ILD is the leading cause of death in SSc, accounting for approximately 35% of SSc-related deaths32–37

Understanding and identifying the risk of mortality in your patients

Footnotes
  • * Estimated time between detection of progressive fibrosis and death. 
  • † Estimates of patients with non-IPF ILD that develop progressive fibrosis based on an online survey of total 486 physicians (243 pulmonologists, 203 rheumatologists and 40 internists) from the United States, Japan, France, Germany, Italy, Spain and the United Kingdom).20
  • CI, confidence interval; DLCO, diffusing capacity of the lung for carbon; FVC, forced vital capacity; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; iNSIP, idiopathic non-specific interstitial; IPF, idiopathic pulmonary fibrosis; NSIP, non-specific interstitial pneumonia; PaO2, partial pressure of oxygen; pSS-ILD, primary Sjögren's syndrome-associated interstitial lung disease; RA, rheumatoid arthritis; RA-ILD, rheumatoid arthritis-associated interstitial lung disease; SSc, systemic sclerosis; SSc-ILD, systemic sclerosis-associated interstitial lung disease; UIP, usual interstitial pneumonia.

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