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Current landscape of IPF and PPF

Understanding pulmonary fibrosis

PF is a complex and devastating disease1-4

IPF and PPF are life-threatening, difficult to diagnose, and devastating for patients and their families.2-5

IPF6-7 is the most common type of chronic, progressive fibrotic interstitial lung disease (ILD).

The cause of IPF is unknown and patients experience significant disease burden and risk of early mortality.

PPF8-11 refers to a subset of non-IPF ILDs characterized by progression—worsening of respiratory symptoms, decline in forced vital capacity (FVC), and increased extent of fibrosis seen in high-resolution computed tomography (HRCT) scans.

Associated etiologies include rheumatoid arthritis (RA), systemic sclerosis (SSc), and hypersensitivity pneumonitis (HP).

Embracing

Patients with connective tissue disease-associated interstitial lung diseases (CTD-ILDs) can develop PPF12,13

ILD overview
ILD overview

The impact of PF is life-changing with significant patient burden14,15

The symptoms of PF alter a patient’s life in many ways, from physical difficulties and increased health risks to detrimental impacts on their mental well-being and quality of life.15-17

Disease burden of IPF and PPF

PF carries a life-threatening prognosis2-4

Deadly indicators of disease progression, such as acute exacerbations, are associated with poor prognosis and a high rate of mortality for patients with IPF or PPF.20,21

Mortality timer
Mortality

Median survival time for IPF is 3 to 5 years4,24

This represents a worse survival rate than many cancers, including breast cancer, prostate cancer, skin cancer, and lymphoma

Group-of-people
Acute exacerbations

~1 in 5 IPF patients25 will experience an acute exacerbation within 3 years of diagnosis

  • Of the patients who are hospitalized for an acute exacerbation, 50% will not survive22

  • Median survival time following an acute exacerbation is 3 to 4 months26

Mortality timer
Mortality

Risk of mortality with PPF varies based on the underlying etiology

  • RA: Patients have a 3x greater risk of death if they develop an ILD27-30

– Median survival time: 3-7 years30,31

  • SSc: More than ⅓ of SSc-related deaths are due to ILD32-37

– Median survival time: ~4 years38,39

  • Chronic hypersensitivity pneumonitis: Median survival time of ~7 years40 

Attention clock
Acute exacerbations

Postexacerbation mortality with PPF ranges from 33% to 83% 21

  • In a Japanese study, 11% of patients with SSc-ILD experienced an acute exacerbation of ILD during the follow-up period, and all resulted in death41

  • In a Danish study, 21% of deaths in patients with RA-ILD were caused by an acute exacerbation of ILD, with the majority occurring within the first year of follow-up29

PPF can have a significant impact on the lives of patients with CTD-ILDs.15

Complexity of diagnosis

Heterogeneity of IPF and PPF makes accurate and prompt diagnosis challenging42

The heterogeneity of IPF and PPF within the patient population, symptom manifestation, and progression makes disease management inherently complex. Further, patients with PPF may have different etiologies, comorbidities, and symptoms. As a result, many patients with IPF or PPF are initially misdiagnosed.43,44

Diseases that can be confused with ILD45

Infectious diseases

  • Tuberculosis46

  • Pneumonia8

Respiratory diseases

  • COPD47-49

  • Asthma49-51

  • Bronchitis52

  • Emphysema49,53

Other conditions

  • Congestive heart failure54

  • Allergies55

 

PF is a common threat across a wide range of ILDs, including CTD-ILDs5

Most patients with CTD-ILDs are at risk for developing PPF at a higher rate than many physicians may realize.12,13 While CTDs differ, common pathogenic pathways to fibrogenesis are shared, irrespective of the trigger for the lung injury.1,3,56-60 The pathogenesis of PPF in CTDs involves the interplay of inflammation and fibrosis early in the course of the disease, making proactive screening essential.1,3,56,57,61

PPF CTD ILD diagram

IPF and PPF are relentless conditions with fibrotic and inflammatory components16,64

The underlying mechanisms driving the progression of PF are complex, and the scientific understanding of them is still evolving. Even with treatment, patients continue to experience irreversible lung damage.1

FVC_or_DLCO_decline
average FVC loss of 
~30 to 35 mL/y65

FVC_or_DLCO_decline
average FVC loss of 
~100 to 200 mL/y66,67

It is essential to employ early and regular monitoring of PF in patients in order to detect progression as soon as possible.61

Disease progression is similar across IPF and PPF7,13

Disease progression is characterized by 7,13 :

Worsening_respiratory_symptoms
Worsening respiratory symptoms
FVC_or_DLCO_decline
FVC or diffusing capacity for carbon monoxide (Dlco) decline
Increasing extent of fibrosis on HRCT
Increasing extent of fibrosis on HRCT

Multidisciplinary teams allow for a holistic approach to management of patients with PF.42

Importance of safeguarding lung function

Disease management remains challenging

When accurate diagnosis is delayed, progression may be more advanced or continue to advance, further complicating disease management.42,43

Disease management
Doctor and Patient

Vigilance is key to protecting patients68,69

Safeguarding lung function remains a top priority to help patients because even small losses can have big impacts.

Keep reading to see how disease management is evolving for patients with IPF or PPF.

Learn More

COPD, chronic obstructive pulmonary disease; CTD, connective tissue disease; CTD-ILD, connective tissue disease- associated interstitial lung disease; DLCO, diffusing capacity of the lungs for carbon monoxide; FVC, forced vital capacity; HP, hypersensitivity pneumonitis; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; IPF, idiopathic pulmonary fibrosis; NSIP, nonspecific interstitial pneumonia pattern; PF, pulmonary fibrosis; PPF, progressive pulmonary fibrosis; RA, rheumatoid arthritis; SSc, systemic sclerosis; UIP, usual interstitial pneumonia.

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