Idiopathic pulmonary fibrosis (IPF), one of the most common interstitial lung diseases, is characterized by an irreversible loss of lung function. Roche’s pirfenidone (Esbriet) was the first disease-modifying therapy (DMT) approved for IPF, followed soon after by Boehringer Ingelheim’s nintedanib (Ofev). These therapies set the stage for fierce competition in the IPF therapy market. We explore this evolving space by examining current medical practice, including the clinical and commercial outlook for pirfenidone and nintedanib; identifying the areas of pressing unmet need in the treatment of IPF; and assessing emerging therapies, including FibroGen’s IV-administered pamrevlumab, Roche’s IV-administered PRM-151, and United Therapeutics’ inhaled treprostinil.
QUESTIONS ANSWERED
How will the size of the IPF population change through 2031? How large are the key subpopulations? What percentage of the IPF population receives drug treatment?
How do interviewed experts view the clinical profiles of Esbriet and Ofev, and what factors drive or constrain their use? What are the most pressing unmet clinical needs in the management of IPF, according to experts?
Which emerging therapies do IPF experts consider most promising? If approved, how would emerging therapies influence the management of IPF and the market position of Ofev and Esbriet? What is the commercial potential of pamrevlumab, PRM-151, and treprostinil?
PRODUCT DESCRIPTION
Niche & Rare Disease Landscape & Forecast provides comprehensive market intelligence with world-class epidemiology, keen insight into current treatment paradigms, in-depth pipeline assessments, and drug forecasts supported by detailed primary and secondary research
SOLUTION ENHANCEMENT
Niche & Rare Disease Landscape & Forecast introduces a new Drug Pipeline chapter with real-time, global pipeline intelligence content directly from Cortellis. This chapter is updated daily and features interactive figures that can be easily downloaded for detailed analysis or presentations.
Regional sales of key therapies to treat IPF: 2021 and 2031
IPF SWOT analysis
Drivers and constraints
What factors are driving the market for IPF?
What factors are constraining the market for IPF?
Drug-class-specific trends
Antifibrotics
Tyrosine kinase inhibitors
Connective tissue growth factor inhibitors
Recombinant human pentraxin-2 proteins
Prostacyclin vasodilators
Forecast
Forecast sales of key therapies in idiopathic pulmonary fibrosis
Etiology and pathophysiology
Disease overview
Etiology
Key risk factors associated with idiopathic pulmonary fibrosis
The lung in IPF
Pathophysiology
Role of inflammation
Pathogenesis of IPF
Disease progression
Characteristics of rapid, moderate, and slow progressors
Disease staging
The GAP index
Symptoms and complications
Symptoms and complications of IPF
Key symptoms and complications of IPF
Key pathways and drug targets
Key pathways and drug targets in IPF
Key pathways and drug targets in IPF
Epidemiology overview
Key findings
Epidemiology populations
Disease definition
Methods and sources used
Number of diagnosed prevalent cases of IPF in the major pharmaceutical markets: 2021-2031
Disease definition
Methods and Sources Used
Number of diagnosed prevalent cases of IPF in the major pharmaceutical markets by GAP severity: 2021-2031
Disease definition
Methods and sources used
Number of diagnosed prevalent cases of IPF in the major pharmaceutical markets by comorbidity: 2021-2031
Sources used for drug-treated prevalent cases of IPF
Number of diagnosed and drug-treated prevalent cases of IPF: 2021-2031
Current treatment overview
Key findings
Diagnosis
Diagnosis of IPF
Diagnostic tests and criteria
Diagnostic criteria for IPF
Comparison of ATS/ERS/JRS/ALAT recommendations for the diagnosis of IPF in 2011 and 2018
Expert insight on diagnosis
Treatment goals
Key endpoints used in clinical trials for IPF
Key current therapies
Overview
Mechanism of action of current drugs used for IPF
Current treatments used for IPF
Market events impacting the use of current therapies in IPF
Clinical trial outcomes for pirfenidone
Key results from select clinical trials investigating pirfenidone for the treatment of IPF
Advantages and disadvantages of pirfenidone
Recent clinical development of pirfenidone
Recent trials of pirfenidone in the treatment of IPF
Expert insight: pirfenidone
Clinical trial outcomes for nintedanib
Key results from select clinical trials investigating nintedanib for the treatment of IPF
Advantages and disadvantages of nintedanib
Ongoing clinical development
Key ongoing trials of nintedanib in the treatment of IPF
Expert insight on nintedanib
Combination of pirfenidone and nintedanib
Clinical trial outcomes for the combination of pirfenidone and nintedanib
Key results from select clinical trials investigating the combination of pirfenidone and nintedanib for the treatment of IPF
Key ongoing trials of the combination of pirfenidone and nintedanib in the treatment of IPF
Expert insight on the combination of pirfenidone and nintedanib
Other medications used to treat IPF
Medical practice
Overview
IPF treatment guidelines
Factors influencing drug selection in IPF
Treatment providers
Treatment initiation
Treatment of severe IPF
Expert insight on approach to treatment
Expert insight on the role of specialty centers for IPF
Generalized treatment decision tree for IPF
Unmet need overview
Current and future attainment of unmet needs in IPF
Top unmet needs in IPF: current and future attainment
Expert insight on the unmet needs in the treatment of IPF
Drug pipeline
Pipeline
Regulatory Milestones
Indication Comparison
Emerging therapies overview
Introduction
Key findings
Key emerging therapies
Estimated launch dates of key emerging therapies for the treatment of IPF
Key ongoing trials of pamrevlumab in the treatment of IPF
Pamrevlumab clinical development
Expert insight: Pamrevlumab
Expectations for launch and sales opportunity of pamrevlumab in IPF
PBI-4050 profile
PBI-4050 clinical development
Expectations for launch and sales opportunity of PBI-4050 in IPF
PRM-151 profile
Key ongoing trials of PRM-151 in the treatment of IPF
PRM-151 clinical development
Expert insight: PRM-151
Expectations for launch and sales opportunity of PRM-151 in IPF
Key ongoing clinical trials of treprostinil (inhaled) in the treatment of IPF
Treprostinil (inhaled) clinical development
Expert insight: treprostinil (inhaled)
Expectations for launch and sales opportunity of treprostinil (inhaled) in IPF
Early-phase pipeline analysis
Select compounds in early-phase development for IPF
Symptomatic or acute therapy in development for IPF
Select symptomatic or acute therapies in early-phase development for IPF
Key discontinuations and failures in IPF
Key development discontinuations and failures
Patient registries
Patient registries for IPF
Prominent patient organizations
Orphan drug designation
Access and reimbursement overview
Region-specific reimbursement practices
Key market access considerations in IPF: United States
General reimbursement environment: United States
Key market access considerations in IPF: EU5
General reimbursement environment: EU5
Appendix
Key abbreviations related to IPF
Brands, marketers, and generic availability of key therapies for IPF by market
IPF bibliography
Anwesh Panda
Anwesh Panda, M.Tech., is an analyst on the Infectious, Niche, & Rare Diseases (INRD) team at Clarivate. In this role, he conducts primary and secondary research on a range of indications. Prior to joining Clarivate, he was a market research analyst at Infiniti Research and Grand View Research. He holds a master’s degree in biotechnology from the Kiit School of Biotechnology in Odisha, India.
Mohani Latwal
Mohani Latwal, B.P.T., M.P.H., Epidemiologist, Epidemiology. Prior to joining Clarivate, she worked as a deputy manager at an NGO. She received her bachelor’s degree in physiotherapy from Delhi University and her master’s degree in public health with a specialization in social epidemiology from the Tata Institute of Social Sciences in Mumbai.