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Acute exacerbation of ILD

Acute exacerbation of ILD is a deadly threat that could strike at any time1–5

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Acute exacerbation of ILD, is characterized by rapid respiratory deterioration (marked increase in dyspnea and hypoxemia)1

As an unpredictable, serious life-threatening event, acute exacerbation of ILD can occur at any time during the disease course of ILDs.1 Acute exacerbation of ILD is associated with a poor prognosis and high rate of mortality1

ACUTE EXACERBATION OF IPF: UNDERSTANDING ITS RISK AND SURVIVAL IMPACT

As many as 1 in 5 IPF patients annually are at risk of a deadly acute exacerbation of IPF.6

Poor survival following an acute exacerbation of IPF7

Acute exacerbation of IPF leads to poor survival

Reprinted from Annals of the American Thoracic Society, 183(4), Ley et al, Clinical course and prediction of survival in idiopathic pulmonary fibrosis, 431-440, ©2022.
Among the 163 patients with rapid deterioration (RD), 90 patients had acute exacerbation of IPF as the most frequent cause of RD, followed by infection (51 patients).2
Comparison of survival curves (from rapid deterioration to death or last follow-up) between IPF patients with acute exacerbation of IPF and infection.2

In patients with IPF, acute exacerbation of IPF and decline in FVC are associated with increased mortality8

Icon presenting that prognosis after acute exacerbation of IPF survival rate is around 3-4 months.

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Survival is poor following an acute exacerbation of IPF with a median prognosis of 3–4 months2,9

Icon demonstrating that 50% of hospitalizations of IPF are caused by acute exacerbation.

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50% of patients who are hospitalized due to an acute exacerbation of IPF die in hospital2

Poor survival following acute exacerbation of IPF2

Poor survival following acute exacerbation of IPF comparable to infection

Reproduced with permission of the © ERS 2021. European Respiratory Journal Feb 2011, 37(2)356-363; DOI: 10.1183/09031936.00159709.
Among the 163 patients with rapid deterioration (RD), 90 patients had acute exacerbation (AE) as the most frequent cause of RD, followed by infection (51 patients).2
Comparison of survival curves (from rapid deterioration to death or last follow-up) between IPF patients with acute exacerbation and infection.2

Comparative risks of death due to acute exacerbation of IPF and categorical declines in FVC % predicted8

Risks of death due to declines in FVC % predicted and acute exacerbation of IPF by hazard ratio

Reprinted from Annals of the American Thoracic Society, 14(9), Paterniti et al, Acute Exacerbation and Decline in Forced Vital Capacity Are Associated with Increased Mortality in Idiopathic Pulmonary Fibrosis, 1395–1402, ©2022
Adjusted HR and 95% CI for death for decline in FVC % predicted (%p-FVC) and acute exacerbations.
Comparisons were made to reference level for baseline lung function (FVC % predicted) and longitudinal variables (decline in FVC % predicted, <5, no exacerbation). *Longitudinal variables were treated as time-varying covariates in the Cox proportional hazards model.
Comparison of survival curves (from initial diagnosis to death or last follow-up) for IPF patients with no rapid deterioration, focal rapid deterioration, bilateral infection and acute exacerbation.2

POST-EXACERBATION MORTALITY OF NON-IPF ILDs

Post-exacerbation mortality in ILDs is reported to range from 33% to 83%.1 Hospital mortality rates in patients with HP range from 75% to 100%1

In a South Korean study, 17% of patients with RA-ILD experienced acute exacerbation of ILD over 33 months in a retrospective review of 84 patients with RA-ILD3

Icon demonstrating that progressive phenotype develops in around 21% of HP patients

In a Danish study, 21% (8/38) of deaths in RA-ILD were caused by acute exacerbation of ILD with the majority of these occurring in the first year of follow-up10

Comparison of the survival curves among RA-ILD patients with UIP pattern on HRCT with different follow-up courses3

Survival of different follow-up courses, including acute exacerbation of ILD, of RA-ILD patients with UIP pattern on HRC

Comparison of the survival curves among RA-ILD patients with UIP pattern on HRCT with different follow-up courses3
Improved (n=5), stable (n=37), progressed (n=28), acute exacerbation of ILD (n=14).
Improvement and progression were defined as >10% respective change in FVC and/or >15% change in DLco.
Adapted from: Song JW, et al. Sarcoidosis Vasc Diffuse Lung Dis. 2013;30:103–112.

How can you assess, approach, and manage the risk of acute exacerbation of ILD?

Footnotes
  • CI, confidence interval; DLCO, diffusing capacity of the lung for carbon; FVC, forced vital capacity; HP, hypersensitivity pneumonitis; HR, hazard ratio; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; IPF, idiopathic pulmonary fibrosis; RA-ILD, rheumatoid arthritis-associated interstitial lung disease; UIP, usual interstitial pneumonia.

  1. Kolb M, Bondue B, Pesci A, et al. Acute exacerbations of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):pii:180071. 

  2. Song JW, Hong S-B, Lim C-M, et al. Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome. Eur Respir J. 2011;37(2):356–363.  

  3. Song JW, Lee HK, Lee CK, et al. Clinical course and outcome of rheumatoid arthritis-related usual interstitial pneumonia. Sarcoidosis Vasc Diffuse Lung Dis. 2013;30(2):103-112.
  4. Tomiyama F, Watanabe R, Ishii T, et al. High Prevalence of Acute Exacerbation of Interstitial Lung Disease in Japanese Patients with Systemic Sclerosis. Tohoku J. Exp. Med. 2016;239, 297–305.
  5. Okamoto M, Fujimoto K, Sadohara J, et al. A retrospective cohort study of outcome in systemic sclerosis-associated interstitial lung disease. Respiratory Investigation. 2016;54, 445–453. 
  6. Ryerson C, Cottin V, Brown K, Collard H. Acute exacerbation of idiopathic pulmonary fibrosis: shifting the paradigm. Eur Respir J. 2015;46(2):512–520.
  7. Ley B, Collard HR, King TE Jr. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;183(4):431–440.
  8. Paterniti MO, Bi Y, Reki D, et al. Acute exacerbation and decline in forced vital capacity are associated with increased mortality in idiopathic pulmonary fibrosis. Ann Am Thorac Soc. 2017;14(9):1395–1402.
  9. Collard HR, Ryerson CJ, Corte TJ, et al. Acute exacerbation of idiopathic pulmonary fibrosis: an international working group report. Am J Respir Crit Care Med. 2016;194(3):265–275.
  10. Hyldgaard C, Ellingsen T, Hilberg O, et al. Rheumatoid arthritis-associated interstitial lung disease: clinical characteristics and predictors of mortality. Respiration. 2019;98(5):455–460.
  11. Flaherty KR, Brown KK, Wells AU, et al. Design of the PF-ILD trial: a double-blind, randomised, placebo-controlled phase III trial of ... in patients with progressive fibrosing interstitial lung disease. BMJ Open Resp Res. 2017;4(1):e000212.
  12. Cottin V, Hirani N, Hotchkin D, et al. Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):180076. 
  13. Maher TM, Molina-Molina M, Russell AM, et al. Unmet needs in the treatment of idiopathic pulmonary fibrosis-insights from patient chart review in five European countries. BMC Pulm Med. 2017;17(1):124.
  14. Molina-Molina M, Aburto M, Acosta O, et al. Importance of early diagnosis and treatment in idiopathic pulmonary fibrosis. Exp Rev Resp Med. 2018;12(7):537–539.
  15. Robalo-Cordeiro C, Campos P, Carvalho L, et al. Idiopathic pulmonary fibrosis in the era of antifibrotic therapy: Searching for new opportunities grounded in evidence. Rev Port Pneumol. 2017;23(5):287–293.

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