Idiopathic pulmonary fibrosis (IPF) is a chronic lung disorder 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 the 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 United Therapeutics’ inhaled treprostinil and Boehringer Ingelheim's PO-administered BI-1015550.
Questions answered
How will the size of the IPF population change through 2032? 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 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 positions of Ofev and Esbriet? What is the commercial potential of inhaled treprostinil and BI-1015550?
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
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Regional sales of key therapies to treat IPF: 2022 and 2032
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
Prostacyclin vasodilators
Phosphodiesterase 4B inhibitors
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
Key findings
Epidemiology populations
Disease definition
Methods and sources used for prevalence of IPF
Diagnosed prevalent cases of IPF: 2022-2032
Disease definition
Methods and sources used for severity stages of IPF
Diagnosed prevalent cases of IPF by GAP severity: 2022-2032
Disease definition
Methods and sources used for comorbidities of IPF
Diagnosed prevalent cases of IPF by comorbidity: 2022-2032
Sources used for drug-treated prevalent cases of IPF
Number of diagnosed and drug-treated prevalent cases of IPF: 2022-2032
Current Treatment
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, 2018, and 2022
Expert insight: diagnosis
Treatment goals
Key endpoints used in clinical trials for IPF
Expert Insight: treatment goals of 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
Advantages and disadvantages of pirfenidone
Key results from select clinical trials investigating pirfenidone for the treatment of IPF
Recent clinical development of pirfenidone
Recent trials of pirfenidone in the treatment of IPF
Expert insight: pirfenidone
Clinical trial outcomes for nintedanib
Advantages and disadvantages of nintedanib
Key results from select clinical trials investigating nintedanib for the treatment of IPF
Recent clinical development of nintedanib
Recent trials of nintedanib in the treatment of IPF
Expert insight: 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: combination of pirfenidone and nintedanib
Expert insight: other medications used to treat IPF
Medical practice
Overview
Factors influencing drug selection in IPF
Treatment providers
Treatment initiation
Treatment of severe IPF
Expert insight: approach to treatment
Expert insight: 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: unmet need in IPF
Drug Pipeline
Pipeline
Regulatory Milestones
Indication Comparison
Emerging Therapies
Key findings
Key emerging therapies
Estimated launch dates of key emerging therapies for the treatment of IPF
Analysis of the clinical development program for treprostinil (inhaled)
Expert insight: treprostinil (inhaled)
Expectations for launch and sales opportunity of treprostinil (inhaled) in IPF
BI-1015550 profile
Key ongoing trials of BI-1015550 in the treatment of IPF
Analysis of the clinical development program for BI-1015550
Expert insight: BI-1015550
Expectations for launch and sales opportunity of BI-1015550 in IPF
Key trials of pamrevlumab in the treatment of IPF
Analysis of the clinical development program for pamrevlumab
Expectations for launch and sales opportunity of Pamrevlumab in IPF
PRM-151 profile
Key results of select clinical trials investigating PRM-151 for the treatment of IPF
Analysis of the clinical development program for PRM-151
Expectations for launch and sales opportunity of PRM-151 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 discontinuations and failures
Patient registries
Patient registries for IPF
Prominent patient organizations
Orphan Drug designation
Access & 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
Abbreviations
IPF bibliography
Shreaa LNU
Shreaa, M.B.A., is an associate healthcare and data analyst on the Infectious, Niche, and Rare Diseases team at Clarivate. She conducts secondary and primary market research on U.S. and European biopharmaceutical markets. She holds an M.B.A. in pharmaceutical management from Jamia Hamdard in New Delhi.