1. Key Insights 2. Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) Market Overview at a Glance 2.1. Market Share (%) Distribution of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) in 2017 2.2. Market Share (%) Distribution of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) in 2028 3. Progressive-Fibrosing Interstitial Lung Disease (PF-ILD): Disease Background and Overview 3.1. Introduction 3.2. Types of ILDs progressing into Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) 3.2.1. Idiopathic Pulmonary Fibrosis (IPF) 3.2.2. Chronic Hypersensitivity Pneumonitis (HP) 3.2.3. Idiopathic Nonspecific Interstitial Pneumonia (INSIP) 3.2.4. Unclassifiable Idiopathic Interstitial Pneumonias (unclassifiable IIPs) 3.2.5. Autoimmune Interstitial Lung Diseases (CTD-ILDs) 3.2.6. Chronic Sarcoidosis 3.2.7. Exposure-related ILDs 3.3. Pathogenesis of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) 3.4. Predictors of Disease Progression 3.5. Diagnosis of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) 3.6. Diagnostic Algorithm of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) 4. Epidemiology and Patient Population 4.1. Key Findings 4.2. Forecast Methodology 4.3. 7MM Total Prevalent Patient Population of Interstitial Lung Disease (ILD) 4.4. 7MM Subtype-specific Prevalence of Interstitial Lung Disease (ILD) 4.5. 7MM Total Prevalent Patient Population of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) 4.6. 7MM Total Prevalent Patient Population of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) by subtypes 5. Country Wise-Epidemiology of PF-ILD 5.1. United States Epidemiology 5.1.1. Assumptions and Rationale 5.1.2. Total Prevalent Cases of Interstitial Lung Disease (ILD) in the United States 5.1.3. Type-Specific Prevalence of Interstitial Lung Disease (ILD) in the United States 5.1.4. Subtype-Specific Prevalence of Interstitial Lung Disease (ILD) in the US 5.1.5. Prevalence of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) in the United States 5.2. EU5 Epidemiology 5.2.1. Germany 5.2.1.1. Assumptions and Rationale 5.2.1.2. Total Prevalent Cases of Interstitial Lung Disease (ILD) in Germany 5.2.1.3. Type-Specific Prevalence of Interstitial Lung Disease (ILD) in Germany 5.2.1.4. Subtype-Specific Prevalence of Interstitial Lung Disease (ILD) in Germany 5.2.1.5. Prevalence of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) in Germany 5.2.2. France 5.2.2.1. Assumptions and Rationale 5.2.2.2. Total Prevalent Cases of Interstitial Lung Disease (ILD) in France 5.2.2.3. Type-Specific Prevalence of Interstitial Lung Disease (ILD) in France 5.2.2.4. Subtype-Specific Prevalence of Interstitial Lung Disease (ILD) in France 5.2.2.5. Prevalence of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) in France 5.2.3. Italy 5.2.3.1. Assumptions and Rationale 5.2.3.2. Total Prevalent Cases of Interstitial Lung Disease (ILD) in Italy 5.2.3.3. Type-Specific Prevalence of Interstitial Lung Disease (ILD) in Italy 5.2.3.4. Subtype-Specific Prevalence of Interstitial Lung Disease (ILD) in Italy 5.2.3.5. Prevalence of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) in Italy 5.2.4. Spain 5.2.4.1. Assumptions and Rationale 5.2.4.2. Total Prevalent Cases of Interstitial Lung Disease (ILD) in Spain 5.2.4.3. Type-Specific Prevalence of Interstitial Lung Disease (ILD) in Spain 5.2.4.4. Subtype-Specific Prevalence of Interstitial Lung Disease (ILD) in Spain 5.2.4.5. Prevalence of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) in Spain 5.2.5. United Kingdom 5.2.5.1. Assumptions and Rationale 5.2.5.2. Total Prevalent Cases of Interstitial Lung Disease (ILD) in the United Kingdom 5.2.5.3. Type-Specific Prevalence of Interstitial Lung Disease (ILD) in the United Kingdom 5.2.5.4. Subtype-Specific Prevalence of Interstitial Lung Disease (ILD) in the United Kingdom 5.2.5.5. Prevalence of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) in the United Kingdom 5.3. Japan Epidemiology 5.3.1. Assumptions and Rationale 5.3.2. Total Prevalent Cases of Interstitial Lung Disease (ILD) in Japan 5.3.3. Type-Specific Prevalence of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) in Japan 5.3.4. Subtype-Specific Prevalence of Interstitial Lung Disease (ILD) in Japan 5.3.5. Prevalence of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) in Japan 6. Treatment and Management of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) 6.1. Treatment Guidelines for Progressive-Fibrosing Lung Disease (PD-ILD) 6.1.1. ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis 6.1.2. EULAR Recommendations for the Treatment of Systemic Sclerosis (SSc-ILD) 6.1.3. German Guideline for Idiopathic Pulmonary Fibrosis (IPF-ILD) 6.1.4. France Practical Guidelines for the Diagnosis and Management of Idiopathic Pulmonary Fibrosis (IPF-ILD) 7. Unmet Needs 8. Emerging Drugs 8.1. Nintedanib: Boehringer Ingelheim 8.1.1. Product Description 8.1.2. Other Developmental Activities 8.1.3. Clinical Development 8.1.4. Clinical Trials Information 8.1.5. Safety and Efficacy 8.1.6. Product Profile 8.2. Pirfenidone: Roche 8.2.1. Product Description 8.2.2. Other Developmental Activities: 8.2.3. Clinical Development 8.2.4. Clinical Trials Information 8.2.5. Safety and Efficacy 8.2.6. Product Profile 9. Progressive-fibrosing interstitial lung disease (PF-ILD): 7 Major Market Analysis 9.1. Key Findings 9.2. Market Size of Progressive-fibrosing interstitial lung disease (PF-ILD) in the 7MM 9.3. Market Size of Progressive-fibrosing interstitial lung disease (PF-ILD) by Therapies in the 7MM 10. Market Outlook: 7MM 10.1. United States Market Size 10.1.1. The total Market size of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) 10.1.2. Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) Market by Current Treatment and Emerging Therapies 10.2. EU5 10.2.1. Germany 10.2.1.1. Total Market size of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) 10.2.1.2. Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) Market by Current Treatment and Emerging Therapies 10.2.2. France 10.2.2.1. Total Market size of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) 10.2.2.2. Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) Market by Current Treatment & Emerging Therapies 10.2.3. Italy 10.2.3.1. Total Market size of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) 10.2.3.2. Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) Market by Current Treatment & Emerging Therapies 10.2.4. Spain 10.2.4.1. Total Market size of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) 10.2.4.2. Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) Market by Current Treatment and Emerging Therapies 10.2.5. United Kingdom 10.2.5.1. Total Market size of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) 10.2.5.2. Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) Market by Current Treatment and Emerging Therapies 10.3. Japan 10.3.1. Total Market size of Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) 10.3.2. Progressive-Fibrosing Interstitial Lung Disease (PF-ILD) Market by Current Treatment & Emerging Therapies 11. Market Drivers 12. Market Barriers 13. SWOT Analysis 14. Appendix 14.1. Report Methodology 15. DelveInsight Capabilities 16. Disclaimer 17. About DelveInsight

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 (2017–2028) 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) 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 (2017–2028) Figure 47 Market Size of PF-ILD by Therapies in the 7MM, in USD Million (2017–2028) 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

Boehringer Ingelheim Roche