ILD patient cases

Fibrotic ILDs in patients like Deborah, Liz and Simon demand vigilance

ILD patients with the HRCT scans in their hands.

Explore typical clinical cases of fibrotic ILDs and their impacts on patients

Icon of Deborah, chronic fibrotic HP patient with progressive phenotype.

Deborah

Chronic fibrotic HP 1 year ago, with progressive phenotype

Meet Deborah
Icon of Liz, sarcoidosis-associated ILD patient with progressive phenotype

Liz

Sarcoidosis-associated ILD 1 year ago, with progressive phenotype

Meet Liz
Icon of Simon, Fibrotic iNSIP 2 patient with progressive phenotype

Simon

Fibrotic iNSIP 2 years ago, with a progressive phenotype

Meet Simon
Female patient with fibrotic cHP

MEET DEBORAH*

Deborah was diagnosed with chronic fibrotic hypersensitivity pneumonitis one year ago.

Age: 40
Pulmonary manifestations: Chronic fibrotic HP 1 year ago, with progressive phenotype
Treatment history: Anti-inflammatory

Despite treatment, Deborah has started losing weight and been suffering from worsening dyspnea,1 and activities that she used to enjoy like swimming have become difficult.

“Since my lung diagnosis, I’ve noticed my breathlessness getting worse. I don’t like my friends to see me like this, so make excuses not to go out”

Patients with chronic fibrotic HP, like Deborah, are predicted to have a significant decline in FVC %, compared with HP patients without fibrosis (HR=0.22; 95% CI=0.10, 0.51; p<0.001)2

HP patients with non-honeycomb fibrosis have a predicted median survival of >7.95 years, compared to 2.76 years in HP patients with honeycomb fibrosis2

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

A progressive fibrosing ILD phenotype develops in an estimated 21% of patients with HP3

Icon demonstrating that an FVC percentage decline of ≥10% is predicted in the first 6–12 months after diagnosis in patients with chronic fibrotic HP.

In patients with chronic fibrotic HP, an FVC percentage decline of ≥10% predicted in the first 6–12 months after diagnosis is associated with increased all-cause mortality1

HOW LONG COULD PATIENTS LIKE DEBORAH SURVIVE? 

Cox model-based survival estimates by clinical diagnosis and radiologic phenotype group2

Lower probability of survival in IPF and HP patients with honeycombing on HRCT compared with no honeycombing

Adapted from Sailsbury ML, et al. Chest. 2019;155(4):699–711.

Kaplan-Meier survival in corticosteroid-treated patients with fibrotic HP vs. never-treated patients4

Lower survival for untreated patients with fibrotic HP compared with patients treated with corticosteroids

Adapted from De Sadeleer LJ, et al. J Clin Med. 2018;8(1):pii:E14.

†  The use of corticosteroid in patients with fibrotic HP resulted in a trend towards a worse survival outcome (HR=2.2; p=0.096)

Icon of down graph symbol illustrating seriousness in patients with chronic fibrotic HP.

Acute exacerbation of ILD is a serious event in patients with chronic fibrotic HP

In patients with chronic fibrotic HP, 75%–100% of patients hospitalized for an acute exacerbation of ILD die in hospital of the event5

Footnotes
  • * Hypothetical patient. Patient quotes for illustrative purposes only.
  • Estimates of the percentage of patients with non-IPF ILD that develop progressive fibrosis based on an online survey of 486 physicians (243 pulmonologists, 203 rheumatologists and 40 internists) from the United States, Japan, France, Germany, Italy, Spain and the United Kingdom.3 
Female patient with sarcoidosis-associated interstitial lung disease

MEET LIZ*

Liz was diagnosed with sarcoidosis-associated interstitial lung disease one year ago.

Age: 55
Underlying diagnosis: Sarcoidosis 6 years ago
Pulmonary manifestations: Sarcoidosis-associated interstitial lung disease 1 year ago, with progressive phenotype
Treatment history: Anti-inflammatory discontinued after 6 months;6,7 antihypertensive

Liz has recently been experiencing worsening symptoms of dyspnea that leaves her feeling tired all the time.6,7 She is not too debilitated yet, but her symptoms are worsening.

“Just walking the dogs leaves me breathless. I worry how long I can keep going before I have let someone else take over. It’s so depressing to be like this”

Patients who develop stage IV pulmonary sarcoidosis, like Liz, have higher rates of death than the general population, with 75% of mortality due to respiratory causes.6

Icon presenting that up to 20% of patients with sarcoidosis-associated ILD develops a fibrosing interstitial lung disease phenotype

A progressive fibrosing interstitial lung disease phenotype develops in up to 20% of patients with sarcoidosis-associated interstitial lung disease3‡

HOW LONG COULD PATIENTS LIKE LIZ SURVIVE?

Survival in patients with stage IV pulmonary sarcoidosis vs. a matched French general population (n=142)6

Lower survival in patients with stage IV pulmonary sarcoidosis compared with matched general population

Adapted from Nardi A, et al. Eur Respir J. 2011;38:1368–1373.

WHAT IS THE IMPACT OF ACUTE WORSENING OF FIBROTIC ILDs IN PATIENTS LIKE LIZ?

Acute worsening§ of pulmonary sarcoidosis in patients with fibrotic sarcoidosis is a serious event and can result in significant pulmonary morbidity.8

73% of patients (94 of 129) with fibrotic sarcoidosis reported two or more acute worsening events over the past year8

Footnotes
  • * Hypothetical patient. Patient quotes for illustrative purposes only.
  • With evidence of upper lobe volume loss with hilar retraction with or without masses, coarse linear bands, honeycombing, bullae and emphysema.6
  • Estimates of the percentage of patients with non-IPF ILD that develop progressive fibrosis based on an online survey of 486 physicians (243 pulmonologists, 203 rheumatologists and 40 internists) from the United States, Japan, France, Germany, Italy, Spain and the United Kingdom.3
  • § Causes of acute worsening of pulmonary sarcoidosis include worsening of the underlying sarcoidosis, infection, acute bronchospasm, or extra-pulmonary causes.8
Male patient with fibrotic iNSIP

MEET SIMON*

Simon was diagnosed with idiopathic fibrotic non-specific interstitial pneumonia two years ago.

Age: 70
Pulmonary manifestations: Fibrotic iNSIP 2 years ago, with a progressive phenotype
Treatment history: Anti-inflammatory; pulmonary rehabilitation for 3 months; treatment for osteoarthritis and irritable bowel syndrome

Despite treatment, Simon continues to suffer from worsening dyspnea and a persistent, non-productive cough that keeps him awake at night.9

“You see people scatter when I cough. They think it’s contagious, which really embarrasses me”

Patients like Simon with fibrotic iNSIP are at risk of early death.9

Icon of lungs affected by the extent of fibrosis.

A progressive fibrosing ILD phenotype develops in an estimated 32% of patients with iNSIP3

Icon of lungs with their declined function.

In patients with iNSIP, a decline in FVC percentage predicted at 12 months is associated with increased disease-related mortality9

Icon representing that around 26% of patients with iNSIP die from their condition within 5 years of diagnosis.

Approximately 26% of patients with iNSIP die from their condition within 5 years of diagnosis9

Footnotes
  • * Hypothetical patient. Patient quotes for illustrative purposes only.
  • Estimates of the percentage of patients with non-IPF ILD that develop progressive fibrosis based on an online survey of 486 physicians (243 pulmonologists, 203 rheumatologists and 40 internists) from the United States, Japan, France, Germany, Italy, Spain and the United Kingdom.3

What can you do to help your patients like Deborah, Liz and Simon?

  1. Gimenez A, Storrer K, Kuranishi L, et al. Change in FVC and survival in chronic fibrotic hypersensitivity pneumonitis. Thorax. 2018;73:391–392.

  2. Salisbury ML, Gu T, Murray S, et al. Hypersensitivity pneumonitis: radiologic phenotypes are associated with distinct survival time and pulmonary function trajectory. Chest. 2019;155(4):699–711.

  3. Wijsenbeek M, Kreuter M, Olson A, et al. Progressive fibrosing interstitial lung diseases: current practice in diagnosis and management. Curr Med Res Opin. 2019:35(11):2015–2024.
  4. De Sadeleer LJ, Hermans F, De Dycker E, et al. Effects of corticosteroid treatment and antigen avoidance in a large hypersensitivity pneumonitis cohort: a single-centre cohort study. J Clin Med. 2018;8(1):pII:E14. 
  5. Kolb M, Bondue B, Pesci A, et al. Acute exacerbations of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):pii:180071.
  6. Nardi A, Brillet P-Y, Letoumelin P, et al. Stage IV sarcoidosis: comparison of survival with the general population and causes of death. Eur Respir J. 2011;38:1368–1373.
  7. American Thoracic Society. Statement on Sarcoidosis. Am J Respir Crit Care Med. 1999;160:736–755.
  8. Baughman RP, Lower EE. Frequency of acute worsening events in fibrotic pulmonary sarcoidosis patients. Respiratory Medicine. 2013;107:2009e2013.
  9. Park IN, Jegal Y, Kim DS, et al. Clinical course and lung function change of idiopathic nonspecific interstitial pneumonia. Eur Respir J. 2009;33:68–76.
  10. 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. N, Jegal Y, Kim DS, et al. Clinical course and lung function change of idiopathic nonspecific interstitial pneumonia. Eur Respir J. 2009;33:68–76.
  11. 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.
  12. 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.
  13. 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.
  14. Ryerson CJ, Cayou C, Topp F, et al. Pulmonary rehabilitation improves long-term outcomes in interstitial lung disease: a prospective cohort study. Respir Med. 2014;108(1):203-210.
  15. Kreuter M, Bendstrup E, Russell A, et al. Palliative care in interstitial lung disease: living well. Lancet Respir Med. 2017;5(12):968-980.
  16. Maher TM, Wuyts W. Management of Fibrosing Interstitial Lung Diseases. Adv Ther. 2019;doi:10.1007/s12325-019-00992-9. [Epub ahead of print].
  17. Sgalla G, Cerri S, Ferrari R, et al. Mindfulness-based stress reduction in patients with interstitial lung diseases: a pilot, single-centre observational study on safety and efficacy. BMJ Open Respir Res. 2015;2(1):e000065.

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