Hear the alarming impact of ILD
Progressive pulmonary fibrosis has a wide range of potential negative impacts on patients1
IPF has a worse 5-year survival than many cancers, including breast, prostate, colon, skin cancer, and lymphoma2
Reproduced with permission of the © ERS 2021. European Respiratory Journal 35 (3) 496-504; DOI: 10.1183/09031936.00077309 Published 28 February 2010.
Patients diagnosed with IPF, considered as the most typical progressive fibrosing ILD, have a median survival of 2–5 years3,4
Survival is poor following an acute exacerbation of IPF with a median prognosis of 3–4 months5,6
In addition to IPF, other ILDs can be progressive and associated with early mortality1,7–11
WORSENED RESPIRATORY SYMPTOMS
Progressive fibrosis is associated with worsened respiratory symptoms, irreversible lung function decline, reduced health-related quality of life, and early mortality1,9,12
5-year mortality rates of non-IPF ILDs13–15
5-year mortality rates for RA-ILD and SSc-ILD sourced as midpoint values from ranges for 5-year mortality rates as follows: RA-ILD 5-year mortality rate 35%–39%;13,14 SSc-ILD 5-year mortality rate 10%–18%.15
Patients with non-IPF progressive fibrosing ILD have an estimated survival time of 2.5–4 years*†7
In patients with RA, ILD triples the risk of death16 and accounts for 10%–20% of deaths17–19
ILD is the leading cause of death in SSc, accounting for approximately 35% of SSc-related deaths20–25
PATIENT'S QUALITY OF LIFE
The burden of progressive fibrosing ILD can substantially reduce a patient’s health-related quality of life12
As fibrotic ILD progresses, there may be a negative impact on your patient’s day-to-day quality of life, independence and emotional wellbeing27
In patients with progressive fibrosing ILDs, cough, dyspnea and fatigue limit physical functional capacity, as well as the ability to perform basic activities and participate in everyday life12,27–29
Decreased social activities
In addition to the functional impact, patients with progressive fibrosing ILD may suffer emotional distress, including feelings of:12,27,28,30,31
Loss of independence
Samantha was diagnosed with IPF at the age of 60. She has since learned, in addition to it being incurable, that it is progressive and the prognosis is poor. She suffers from worsening fatigue and depression as IPF progresses. Forced to retire earlier than planned due to worsening disability, she finds that she has unfortunately more time now to dwell on her IPF diagnosis and what it continues to take away from her. She wishes she had spoken up much earlier when she first started noticing symptoms, and wonders whether that could have led to an earlier diagnosis.
“My response to the IPF diagnosis was total disbelief. In the past, I would normally distract myself from bad news like that by immersing myself in my day job. But I have had to call it a day several years earlier than I had planned. Now I am just stuck at home feeling more and more constricted day by day. And I have all the time to think about it, and wonder whether this could have been caught way before if I had just been referred earlier.”
According to a 2020 ILD expert consensus study:
Up to 45.6% of patients suffered fatigue and 27.2% of patients suffered depression as fibrotic ILD progressed26
26.7% of patients retired due to progressive fibrosing ILDs26
48.1% of progressive fibrosing ILD patients had total permanent disability and 22.8% lost their job because of it26
There is an under-recognized interplay between lung diseases such as ILD and depression32
Adapted from: Verma S, et al. N Am J Med Sci. 2014;6(6):240-249. The physical burden, decline in functional capacity, decrease in exercise capacity and decrease in quality of life in ILD can have a cumulative effect making the patient vulnerable to depression.32
Depressive symptoms in ILD are common, persistent, and strongly independently correlated with dyspnea, pain, sleep quality and forced vital capacity (FVC)33
Most patients with ILDs experience fatigue, an important negative predictor of quality of life that has found to be associated with depression and anxiety in these patients34
Early recognition and referral is vital because patients like Samantha may lose independence and quality of life as their fibrotic ILD progresses26,27
- * 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 a total of 486 physicians (243 pulmonologists, 203 rheumatologists and 40 internists) from the United States, Japan, France, Germany, Italy, Spain and the United Kingdom).7
CTD-ILD, connective tissue disease-associated interstitial lung disease; FVC, forced vital capacity; ILD, interstitial lung disease; IPF, idiopathic pulmonary fibrosis; RA, rheumatoid arthritis; RA-ILD, rheumatoid arthritis-associated interstitial lung disease; SSc, systemic sclerosis; SSc-ILD, systemic sclerosis-associated interstitial lung disease.
- Ḳolb M, Vašáková M. The natural history of progressive fibrosing interstitial lung diseases. Respir Res. 2019;20:57.
- Vancheri C, Failla M, Crimi N, Raghu G. Idiopathic pulmonary fibrosis: a disease with similarities and links to cancer biology. Eur Respir J. 2010;35(3):496–504.
- 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.
- Nathan SD, du Bois RM. Idiopathic pulmonary fibrosis trials: recommendations for the jury. Eur Respir J. 2011;38(5):1002–1004.
- Song JW, Hong SB, Lim CM, Koh Y, Kim DS. Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome. Eur Respir J.
- 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.
- 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.
- Cottin V, Wollin L, Fischer A, et al. Fibrosing interstitial lung diseases: knowns and unknowns. Eur Respir Rev. 2019;28(151):pii:180100.
- Maher TM, Wuyts W. Management of fibrosing interstitial lung diseases. Adv Ther. 2019;36(7):1518–1531.
- Wong AW, Ryerson C, Guler S. Progression of fibrosing interstitial lung disease. Respir Res. 2020:29;21(1):32.
- 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.
- Flaherty KR, Brown KK, Wells AU, et al. Design of the PF-ILD trial: A double-blind, randomised, placebo-controlled phase III trial of nintedanib in patients with progressive fibrosing interstitial lung disease. BMJ Open Resp Res. 2017;4(1):e000212.
- Hyldgaard C, Hilberg O, Pedersen AB, et al. A population-based cohort study of rheumatoid arthritis-associated interstitial lung disease: comorbidity and mortality. Ann Rheum Dis. 2017;76(10):1700–1706.
- Raimundo K, Solomon JJ, Olson AL, et al. Rheumatoid arthritis – interstitial lung disease in the United States: prevalence, incidence, and healthcare costs and mortality. J Rheumatol. 2019;46(4):360–369.
- Bouros D, Wells AU, Nicholson AG, et al. Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome.
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- Bongartz T, Nannini C, Medina-Velasquez YF, et al. Incidence and mortality of interstitial lung disease in rheumatoid arthritis: a population-based study. Arthritis Rheum. 2010;62(6):1583–1591.
- Cassone G, Manfredi A, Vacchi C, et al. Treatment of rheumatoid arthritis-associated interstitial lung disease: lights and shadows. J Clin Med. 2020;9(4):1082.
- Olson AL, Swigris JJ, Sprunger DB, et al. Rheumatoid arthritis–interstitial lung disease–associated mortality. Am J Respir Crit Care Med. 2011;183:372–378.
- 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.
- Cappelli S, Bellando Randone S, Camiciottoli G, et al. Interstitial lung disease in systemic sclerosis: where do we stand? Eur Respir Rev. 2015;24;411–419.
- Distler O, Assassi S, Cottin V, et al. Predictors of progression in systemic sclerosis patients with interstitial lung disease. Eur Respir J. 2020;55;1902026.
- Distler O, Volkmann ER, Hoffmann-Vold AM, et al. Current and future perspectives on management of systemic sclerosis-associated interstitial lung disease. Expert Rev Clin Immunol. 2019;15;1009–1017.
- Tyndall AJ, Bannert B, Vonk M, et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010;69(10):1809–1815.
- Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972-2002. Ann Rheum Dis. 2007;66(7):940–944.
- Roofeh D, Jaafar S, Vummidi D, et al. Management of systemic sclerosis-associated interstitial lung disease. Curr Opin Rheumatol. 2019;31;241–249.
- Wuyts WA, Papiris S, Manali E, et al. The burden of progressive fibrosing interstitial lung disease: a DELPHI approach. Adv Ther. 2020;37(7):3246–3264.
- Swigris JJ, Brown KK, Abdulqawi R, et al. Patients’ perceptions and patient-reported outcomes in progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):pii:180075.
- Morisset J, Dubé B, Garvey C, et al. The unmet educational needs of patients with interstitial lung disease: setting the stage for tailored pulmonary rehabilitation. Ann Am Thorac Soc. 2016;13:1026–1033.
- Olson AL, Brown KK, Swigris JJ. Understanding and optimizing health-related quality of life and physical functional capacity in idiopathic pulmonary fibrosis. Patient Relat Outcome Meas. 2016;7:29–35.
- Duck A, Spencer LG, Bailey S, et al. Perceptions, experiences and needs of patients with idiopathic pulmonary fibrosis. J Adv Nurs. 2014;71(5):1055–1065.
- Hinz A, Brähler E, Möde R, et al. Anxiety and depression in sarcoidosis: the influence of age, gender, affected organs, concomitant diseases and dyspnoea. Sarcoidosis Vasc Diffuse Lung Dis. 2012;29(2):139–146.
- Verma S, Cardenas-Garcia J, Mohapatra PR, Talwar A. Depression in pulmonary arterial hypertension and interstitial lung diseases. N Am J Med Sci. 2014;6(6):240–249.
- Ryerson CJ, Arean PA, Berkeley J, et al. Depression is a common and chronic comorbidity in patients with interstitial lung disease. Respirology. 2012;17(3):525–532.
- Kahlmann V, Moor CC, Wijsenbeek MS. Managing fatigue in patients with interstitial lung disease. Chest. 2020;S0012-3692(20)30873–30874.