Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) - Market Insights, Epidemiology, and Market Forecast-2028

Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) - Market Insights, Epidemiology, and Market Forecast-2028 report deliver an in-depth understanding of the disease, historical & forecasted epidemiology as well as the market trends of PF-ILD in the United States, EU5 (Germany, France, Italy, Spain, and the United Kingdom), and Japan.
PF-ILD is a collective term used for indications that share a common progressive phenotype. The phenotype is characterized by worsening respiratory symptoms, lung function decline, limited response to immune-modulatory therapies, decreased quality of life, and potentially, early death. Interstitial lung disease (ILD) encompasses a large group of more than 200 parenchymal pulmonary disorders, of which the majority are classified as rare. ILD is further classifies into various types and subtypes. The major classification includes idiopathic interstitial pneumonia (IIPs), autoimmune ILDs, hypersensitivity pneumonitis (HP), sarcoidosis, and several other ILDs. Further, IIP is classified into idiopathic pulmonary fibrosis (IPF)which contributes to the majority of the cases, iNSIP, unclassifiable IIPs, and others IIPs. Autoimmune ILDs are classified into IPAF, RA-ILD, SSc-ILD, and other autoimmune ILDs.
According to , the patient pool of PF-ILD includes the cases of IPF, iNSIP, unclassifiable IIPs, IPAF, RA-ILD, SSc-ILD, HP, sarcoidosis and other ILDs related to occupational exposures.

Geography Covered
The United States
EU5 (Germany, France, Italy, Spain, and the United Kingdom)
Japan

Study Period: 20172028
Progressive-Fibrosing Interstitial Lung DiseaseDisease Understanding
PF-ILDs is characterized by worsening respiratory symptoms, lung function decline, limited response to immune-modulatory therapies, decreased quality of life, and potentially, early death. Irrespective of the clinical diagnosis, these PF-ILDs show commonalities in the underlying pathogenetic mechanisms that drive a self-sustaining process of pulmonary fibrosis.
Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) Epidemiology
The disease epidemiology covered in the report provides historical as well as forecasted epidemiology (Total Prevalent Cases of Interstitial Lung Disease (ILD) in the 7MM, Type-specific Prevalent Cases of Interstitial Lung Disease (ILD) in the 7MM, Subtype-specific Prevalent Cases of Interstitial Lung Disease (ILD) in the 7MM, and Total Prevalent Cases of PF-ILD in the 7MM) scenario of PF-ILD in the 7MM covering United States, EU5 countries (Germany, France, Italy, Spain, and United Kingdom) and Japan from 20172028.

According to , the total prevalent population of Interstitial Lung Disease in seven major markets was 736,184 in 2017. These cases are expected to increase with a significant CAGR during the study period (20172028).

The total prevalent population of PF-ILD in seven major markets was 228,613 in 2017.

Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) Drug Chapters
Evidence-based treatment for PFILDs patients are currently limited, and often receive corticosteroids and/or off-label immunosuppressive therapy with a variable outcome. Because of commonalities between IPF and PF-ILDs, it has been proposed that the potential efficacy and tolerability of antifibrotic drugs, like pirfenidone and nintedanib, should be evaluated in PF-ILDs. By far, the most commonly used first-line therapy for all the non-IPF fibrosing ILDs was corticosteroids. Because PF-ILDs have been defined by progression despite conventional therapy, there is inherently a substantial unmet need for the treatment of these conditions. Antifibrotic drugs effective to slow down disease progression in IPF may conceivably have comparable efficacy in other PF-ILDs.

Not many companies are working on this patient pool. Currently, two companies, i.e., Boehringer Ingelheim and Roche are investigating their potential molecules to provide treatment of PF-ILD. Nintedanib (Ofev) and Pirfenidone (Esbriet) is under development for PF-ILD. Both the drugs are approved for IPF and are being tested to know whether they can be a potential approach for PF-ILD. Pirfenidone is under development for SSc-ILD as well, but recently, it was reported that it does not stabilize or improve lung function in patients with SSc-ILD. The failure of pirfenidone can be proved a boon for nintedanib, and hence it is expected that nintedanib will generate a major market revenue than pirfenidone.
Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) Market Outlook
According to the total market size of PF-ILDs in seven major markets was USD 233.19 million in 2017. The market size of PF-ILD is expected to increase at a significant CAGR during the study period (20172028).
Among all the seven major markets, the United States accounts for the highest market size of PF-ILD. In 2017, the market size of PF-ILD was found to be USD 158.73 million.

Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) Drugs Uptake
The current market size of PF-ILD is mainly attributed by corticosteroids, mycophenolate mofetil, azathioprine, cyclosporine, tacrolimus, and cyclophosphamide. The market size is expected to increase during the forecast period (20192028), due to potential therapies nintedanib (Ofev) and pirfenidone (Esbriet).

Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) Report Insights
Patient Population by total prevalent cases of ILD
Type-specific prevalent cases of ILD
Subtype-specific prevalent cases of ILD
Prevalent cases of PF-ILD
Therapeutic Approaches
Pipeline Analysis
Market Size and Trends
Market Opportunities
Impact of upcoming Therapies

Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) Report Key Strengths
10 Year Forecast
7MM Coverage
Epidemiology Segmentation
Drugs Uptake
Highly Analyzed Market
Key Cross Competition

Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) Report Assessment
Current Treatment Practices
Unmet Needs
Detailed Pipeline Product Profiles
Market Attractiveness
Market Drivers and Barriers

Key Benefits
This report will help to develop Business Strategies by understanding the trends shaping and driving PF-ILD market
Organize sales and marketing efforts by identifying the best opportunities for PF-ILD market
To understand the future market competition in the PF-ILD market.

List Of Tables

Table 1 Total Prevalent Patient Population of Interstitial Lung Disease (ILD) in 7MM (2017-2028)
Table 2 Subtype-Specific Prevalence of ILD in the United States (2017-2028)
Table 3 Total Prevalent Patient Population of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) in the 7MM (2017-2028)
Table 4 Prevalence of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) by subtypes in the 7MM (2017-2028)
Table 5 Total Prevalent Population of ILD in the United States (2017-2028)
Table 6 Type-Specific Prevalence of ILD in the United States (2017-2028)
Table 7 Subtype-Specific Prevalence of ILD in the United States (2017-2028)
Table 8 Prevalence of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) in the United States (2017-2028)
Table 9 Total Prevalent Population of ILD in Germany (2017-2028)
Table 10 Type-Specific Prevalence of ILD in Germany (2017-2028)
Table 11 Subtype-Specific Prevalence of ILD in Germany (2017-2028)
Table 12 Prevalence of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) in Germany (2017-2028)
Table 13 Total Prevalent Population of ILD in France (2017-2028)
Table 14 Type-Specific Prevalence of ILD in France (2017-2028)
Table 15 Subtype-Specific Prevalence of ILD in France (2017-2028)
Table 16 Prevalence of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) in France (2017-2028)
Table 17 Total Prevalent Population of ILD in Italy (2017-2028)
Table 18 Type-Specific Prevalence of ILD in Italy (2017-2028)
Table 19 Subtype-Specific Prevalence of ILD in Italy (2017-2028)
Table 20 Prevalence of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) in Italy (2017-2028)
Table 21 Total Prevalent Population of ILD in Spain (2017-2028)
Table 22 Type-Specific Prevalence of ILD in Spain (2017-2028)
Table 23 Subtype-Specific Prevalence of ILD in Spain (2017-2028)
Table 24 Prevalence of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) in Spain (2017-2028)
Table 25 Total Prevalent Population of ILD in the United Kingdom (2017-2028)
Table 26 Type-Specific Prevalence of ILD in the UK (2017-2028)
Table 27 Subtype-Specific Prevalence of ILD in the UK (2017-2028)
Table 28 Prevalence of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) in the United Kingdom (2017-2028)
Table 29 Total Prevalent Population of ILD in Japan (2017-2028)
Table 30 Type-Specific Prevalence of ILD in Japan (2017-2028)
Table 31 Subtype-Specific Prevalence of ILD in Japan (2017-2028)
Table 32 Prevalence of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) in Japan (2017-2028)
Table 33 Nintedanib, Clinical Trial Description, 2019
Table 34 Pirfenidone , Clinical Trial Description, 2019
Table 35 Market size of PF-ILD by Region in USD Million (20172028)
Table 36 7MM Market Size of PF-ILD by Current Treatment and Emerging Therapies in USD Million (2017-2028)
Table 37 United States Market Size of PF-ILD in USD Million (2017-2028)
Table 38 The US Market Size of PF-ILD by Current Treatment and Emerging Therapies in USD Million (2017-2028)
Table 39 Germany Market Size of PF-ILD in USD Million (2017-2028)
Table 40 Germany Market Size of PF-ILD by Current Treatment and Emerging Therapies in USD Million (2017-2028)
Table 41 France Market Size of PF-ILD in USD Million (2017-2028)
Table 42 France Market Size of PF-ILD by Current Treatment and Emerging Therapies in USD Million (2017-2028)
Table 43 Italy Market Size of PF-ILD in USD Million (2017-2028)
Table 44 Italy Market Size of PF-ILD by Current Treatment and Emerging Therapies in USD Million (2017-2028)
Table 45 Spain Market Size of PF-ILD in USD Million (2017-2028)
Table 46 Spain Market Size of PF-ILD by Current Treatment and Emerging Therapies in USD Million (2017-2028)
Table 47 United Kingdom Market Size of PF-ILD in USD Million (2017-2028)
Table 48 The UK Market Size of PF-ILD by Current Treatment and Emerging Therapies in USD Million (2017-2028)
Table 49 Japan Market Size of PF-ILD in USD Million (2017-2028)
Table 50 Japan Market Size of PF-ILD by Current Treatment and Emerging Therapies in USD Million (2017-2028)

List Of Figures

Figure 1 ILDs with overlapping area shows the probability of having a progressive-fibrosing phenotype
Figure 2 Known and Unknowns of PF-ILDs
Figure 3 Types of ILD most likely to have a progressive-fibrosing phenotype
Figure 4 Fibrosis of the alveolus wall
Figure 5 Types of Connective tissue disease-associated interstitial lung disease (CTD-ILD)
Figure 6 Concepts in the pathogenesis of RA-ILD
Figure 7 Mechanisms involved in the pathogenesis and progression of fibrosing interstitial lung diseases
Figure 8 Diagnosis of ILDs that may present a progressive phenotype
Figure 9 Total Prevalent Patient Population of Interstitial Lung Disease (ILD) in the 7MM (2017-2028)
Figure 10 Subtype-Specific Prevalence of ILD in the United States (2017-2028)
Figure 11 Total Prevalent Patient Population of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) in the 7MM (2017-2028)
Figure 12 Prevalence of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) by subtypes in the 7MM (2017-2028)
Figure 13 Total Prevalent Cases of ILD in the United States (2017-2028)
Figure 14 Type-Specific Prevalence of ILD in the United States (2017-2028)
Figure 15 Subtype-Specific Prevalence of ILD in the United States (2017-2028)
Figure 16 Prevalence of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) in the United States (2017-2028)
Figure 17 Total Prevalent Cases of ILD in Germany (2017-2028)
Figure 18 Type-Specific Prevalence of ILD in Germany (2017-2028)
Figure 19 Subtype-Specific Prevalence of ILD in Germany (2017-2028)
Figure 20 Prevalence of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) in Germany (2017-2028)
Figure 21 Total Prevalent Cases of ILD in France (2017-2028)
Figure 22 Type-Specific Prevalence of ILD in France (2017-2028)
Figure 23 Subtype-Specific Prevalence of ILD in France (2017-2028)
Figure 24 Prevalence of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) in France(2017-2028)
Figure 25 Total Prevalent Cases of ILD in Italy (2017-2028)
Figure 26 Type-Specific Prevalence of ILD in Italy (2017-2028)
Figure 27 Subtype-Specific Prevalence of ILD in Italy (2017-2028)
Figure 28 Prevalence of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) in Italy (2017-2028)
Figure 29 Total Prevalent Cases of ILD in Spain (2017-2028)
Figure 30 Type-Specific Prevalence of ILD in Spain (2017-2028)
Figure 31 Subtype-Specific Prevalence of ILD in Spain (2017-2028)
Figure 32 Prevalence of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) in Spain (2017-2028)
Figure 33 Total Prevalent Cases of ILD in the United Kingdom (2017-2028)
Figure 34 Type-Specific Prevalence of ILD in the United Kingdom (2017-2028)
Figure 35 Subtype-Specific Prevalence of ILD in the United Kingdom (2017-2028)
Figure 36 Prevalence of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) in the United Kingdom (2017-2028)
Figure 37 Total Prevalent Cases of ILD in Japan (2017-2028)
Figure 38 Type-Specific Prevalence of ILD in Japan (2017-2028)
Figure 39 Subtype-Specific Prevalence of ILD in Japan (2017-2028)
Figure 40 Prevalence of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) in Japan (2017-2028)
Figure 41 Current understanding of the mode of action of nintedanib in fibrotic lung diseases
Figure 42 Unmet Needs
Figure 43 Mechanism of action of Nintedanib
Figure 44 Regulatory Milestones of Nintedanib
Figure 45 Clinical Development of Nintedanib
Figure 46 Market size of PF-ILD by Region in USD Million (20172028)
Figure 47 Market Size of PF-ILD by Therapies in the 7MM, in USD Million (20172028)
Figure 48 United States Market Size of PF-ILD in USD Million (2017-2028)
Figure 49 The US Market Size of PF-ILD by Current Treatment and Emerging Therapies in USD Million (2017-2028)
Figure 50 Germany Market Size of PF-ILD in USD Million (2017-2028)
Figure 51 Germany Market Size of PF-ILD by Current Treatment and Emerging Therapies in USD Million (2017-2028)
Figure 52 France Market Size of PF-ILD in USD Million (2017-2028)
Figure 53 France Market Size of PF-ILD by Current Treatment and Emerging Therapies in USD Million (2017-2028)
Figure 54 Italy Market Size of PF-ILD in USD Million (2017-2028)
Figure 55 Italy Market Size of PF-ILD by Current Treatment and Emerging Therapies in USD Million (2017-2028)
Figure 56 Spain Market Size of PF-ILD in USD Million (2017-2028)
Figure 57 Spain Market Size of PF-ILD by Current Treatment and Emerging Therapies in USD Million (2017-2028)
Figure 58 United Kingdom Market Size of PF-ILD in USD Million (2017-2028)
Figure 59 The UK Market Size of PF-ILD by Current Treatment and Emerging Therapies in USD Million (2017-2028)
Figure 60 Japan Market Size of PF-ILD in USD Million (2017-2028)
Figure 61 Japan Market Size of PF-ILD by Current Treatment and Emerging Therapies in USD Million (2017-2028)
Figure 62 Market Drivers
Figure 63 Market Barriers
Figure 64 Ofev for IPF/SSc-ILD/PF-ILD SWOT analysis

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