Idiopathic Pulmonary Fibrosis - Pipeline Insight, 2024

This “Idiopathic Pulmonary Fibrosis - Pipeline Insight, 2024” report provides comprehensive insights about 80+ companies and 100+ pipeline drugs in Idiopathic Pulmonary Fibrosis pipeline landscape. It covers the pipeline drug profiles, including clinical and nonclinical stage products. It also covers the therapeutics assessment by product type, stage, route of administration, and molecule type. It further highlights the inactive pipeline products in this space.

Geography Covered

• Global coverage

Idiopathic Pulmonary Fibrosis: Understanding

Idiopathic Pulmonary Fibrosis: Overview

Idiopathic pulmonary fibrosis (IPF) is a lung disorder where there is scarring of the lungs from an unknown cause. It is usually a progressive disease with a poor long-term prognosis. The classic features of the disorder include progressive dyspnea and a nonproductive cough. Pulmonary function tests usually reveal restrictive impairment and diminished carbon monoxide diffusing capacity.

Environmental factors like smoking, chronic aspiration, or viral infections, along with advancing age, can lead to respiratory alveolar epithelial injury and are thought to be the likely driving factors for the pathogenesis of IPF. With an epithelial injury, there is an activation of fibroblasts and dysregulated repair of the alveolar epithelium. When this leads to increased matrix deposition in the lung interstitium and scarring, there is a destruction of lung architecture that results in pulmonary fibrosis.[7] The destruction of lung architecture impairs gas exchange and will progress to hypoxic respiratory failure, a hallmark of advanced disease.

The most common presenting symptoms of IPF are dyspnea on exertion and cough, followed by fatigue. The diagnosis is often delayed, as most patients are diagnosed more than a year after symptom onset. Since symptoms are nonspecific and the disease is idiopathic, other conditions must be excluded before diagnosis. History of exposure to inhaled dust, metals, asbestos, mold, or birds should be elicited to exclude other interstitial lung diseases. The diagnosis can be made without biopsy if there are compatible imaging tests, appropriate clinical history, and exclusion of other conditions. The classic pattern of imaging on computed tomography (CT) scan will show a peripheral distribution of bilateral fibrosis, more pronounced at the bases. If there is diagnostic uncertainty, IPF can also be diagnosed by lung biopsy.

Pulmonary function tests every 3 to 6 months should be performed based on symptoms and the disease's progression. However, serial chest imaging is not always necessary. Tools like GAP (gender, age physiology) score issue points for the male gender, advanced age, forced vital capacity, and diffusing capacity or transfer factor of the lung for carbon monoxide and can be used to assess long-term prognosis, with a high GAP score indicating worse mortality. This is mainly used when considering a patient for a lung transplant referral. It is also important to assess the patient's functional status objectively and screen for hypoxic respiratory failure. Most Interstitial Lung Disease specialty centers use the 6-minute walk test to accomplish both. The treatment includes supportive measures, oxygen supplementation when needed, anti-fibrotic drugs, and lung transplants for severe disease.

Idiopathic Pulmonary Fibrosis- Pipeline Insight, 2024 report outlays comprehensive insights of present scenario and growth prospects across the indication. A detailed picture of the Idiopathic Pulmonary Fibrosis pipeline landscape is provided which includes the disease overview and Idiopathic Pulmonary Fibrosis treatment guidelines. The assessment part of the report embraces, in depth Idiopathic Pulmonary Fibrosis commercial assessment and clinical assessment of the pipeline products under development. In the report, detailed description of the drug is given which includes mechanism of action of the drug, clinical studies, NDA approvals (if any), and product development activities comprising the technology, Idiopathic Pulmonary Fibrosis collaborations, licensing, mergers and acquisition, funding, designations and other product related details.

Report Highlights

• The companies and academics are working to assess challenges and seek opportunities that could influence Idiopathic Pulmonary Fibrosis R&D. The therapies under development are focused on novel approaches to treat/improve Idiopathic Pulmonary Fibrosis.

Idiopathic Pulmonary Fibrosis Emerging Drugs Chapters

This segment of the Idiopathic Pulmonary Fibrosis report encloses its detailed analysis of various drugs in different stages of clinical development, including phase II, I, preclinical and Discovery. It also helps to understand clinical trial details, expressive pharmacological action, agreements and collaborations, and the latest news and press releases.

Idiopathic Pulmonary Fibrosis Emerging Drugs

Pamrevlumab: FibroGenPamrevlumab is a proprietary therapeutic antibody developed by FibroGen to inhibit the activity of connective tissue growth factor (CTGF), a common factor in chronic fibrotic and proliferative disorders characterized by persistent and excessive scarring that can lead to organ dysfunction and failure. Pamrevlumab represents a potential treatment for a broad array of fibrotic and proliferative disorders that affect organ systems throughout the body. Currently, the drug is in the Phase III stage of its development for the treatment of Idiopathic Pulmonary Fibrosis.

Tipelukast: MediciNovaMN-001 (tipelukast) is a novel, orally bioavailable small molecule compound which exerts its effects through several mechanisms to produce its anti-fibrotic and anti-inflammatory activity in preclinical models, including leukotriene (LT) receptor antagonism, inhibition of phosphodiesterases (PDE) (mainly 3 and 4), and inhibition of 5-lipoxygenase (5-LO). The 5-LO/LT pathway has been postulated as a pathogenic factor in fibrosis development and MN-001’s inhibitory effect on 5-LO and the 5-LO/LT pathway is considered to be a novel approach to treat fibrosis. MN-001 has been shown to down-regulate expression of genes that promote fibrosis including LOXL2, Collagen Type 1 and TIMP-1. MN-001 has also been shown to down-regulate expression of genes that promote inflammation including CCR2 and MCP-1. In addition, histopathological data shows that MN-001 reduces fibrosis in multiple animal models. The US Food and Drug Administration (FDA) has granted orphan-drug designation to MN-001 (tipelukast) for treatment of idiopathic pulmonary fibrosis (IPF). Currently, the drug is in the Phase II stage of its development for the treatment of IPF.

PLN-74809: Pliant TherapeuticsPLN-74809 is an oral small-molecule dual-selective inhibitor of avß6 and avß1 integrins for the treatment of IPF. While present at very low levels in healthy tissues, these integrins are upregulated in the lungs of IPF patients where they activate TGF-ß, a key driver of the fibrotic process. Blocking these integrins is designed to stop TGF-ß activation, potentially halting the growth of scar tissue. PLN-74809 has received Fast Track Designation and Orphan Drug Designation from the US Food and Drug Administration (FDA) in IPF and Orphan Drug Designation from the European Medicines Agency (EMA) in IPF. The company has completed enrollment in the Phase 2a clinical trial of PLN-74809 in patients with idiopathic pulmonary fibrosis (IPF) and announced positive data from the trial.

HZN-825: Horizon PharmaceuticalsHZN-825 is an oral selective LPAR1 antagonist that has shown early signs of clinical impact in systemic sclerosis. LPAR1 signaling has been implicated in fibrosis and inflammation, and preclinical and clinical evidence support the antifibrotic potential of LPAR1 antagonism across multiple organ systems, including both lung and skin. Currently, the drug is in the Phase II stage of its development for the treatment of Idiopathic Pulmonary Fibrosis.

Brilaroxazine: Reviva PharmaceuticalsBrilaroxazine, a novel serotonin-dopamine modulator with multifaceted activities has the potential to treat idiopathic pulmonary fibrosis (IPF). Serotonin (5-HT) signaling plays a key role, via 5-HT2A/2B/7 receptors, in the vasoactive effect on pulmonary arteries and lung myofibroblast actions. Brilaroxazine displays a high affinity and functional activity for the 5-HT2A/2B/7 receptors and moderate affinity for the serotonin transporter. Brilaroxazine’s effects on vascular fibrosis (5-HT2B receptor), proliferation (5-H2A/2B receptor), relaxation (5-HT2A receptor), inflammation (5-HT7 receptor), and pro-inflammatory cytokines have created interest in the potential to treat IPF. Brilaroxazine was evaluated in a bleomycin (BLM)-induced rat model of IPF receiving either brilaroxazine 15 mg twice daily for 21 days starting at day 1 (BT) or at day 10 (BI) and demonstrated efficacy with significant improvements in key endpoints in the bleomycin (BLM)-induced rat model of IPF. Currently, the drug is in the Phase I stage of its development for the treatment of IPF.

Idiopathic Pulmonary Fibrosis: Therapeutic Assessment

This segment of the report provides insights about the different Idiopathic Pulmonary Fibrosis drugs segregated based on following parameters that define the scope of the report, such as:

Major Players in Idiopathic Pulmonary Fibrosis

• There are approx. 80+ key companies which are developing the therapies for Idiopathic Pulmonary Fibrosis. The companies which have their Idiopathic Pulmonary Fibrosis drug candidates in the most advanced stage, i.e. phase III include, FibroGen.

Phases

This report covers around 100+ products under different phases of clinical development like

• Late stage products (Phase III)
• Mid-stage products (Phase II)
• Early-stage product (Phase I) along with the details of
• Pre-clinical and Discovery stage candidates
• Discontinued & Inactive candidates

Route of Administration

Idiopathic Pulmonary Fibrosis pipeline report provides the therapeutic assessment of the pipeline drugs by the Route of Administration. Products have been categorized under various ROAs such as

• Oral
• Intravenous
• Subcutaneous
• Parenteral
• Topical

Molecule Type

Products have been categorized under various Molecule types such as

• Recombinant fusion proteins
• Small molecule
• Monoclonal antibody
• Peptide
• Polymer
• Gene therapy

Product Type

Drugs have been categorized under various product types like Mono, Combination and Mono/Combination.

Idiopathic Pulmonary Fibrosis: Pipeline Development Activities

The report provides insights into different therapeutic candidates in phase II, I, preclinical and discovery stage. It also analyses Idiopathic Pulmonary Fibrosis therapeutic drugs key players involved in developing key drugs.

Pipeline Development Activities

The report covers the detailed information of collaborations, acquisition and merger, licensing along with a thorough therapeutic assessment of emerging Idiopathic Pulmonary Fibrosis drugs.

Idiopathic Pulmonary Fibrosis Report Insights

• Idiopathic Pulmonary Fibrosis Pipeline Analysis
• Therapeutic Assessment
• Unmet Needs
• Impact of Drugs

Idiopathic Pulmonary Fibrosis Report Assessment

• Pipeline Product Profiles
• Therapeutic Assessment
• Pipeline Assessment
• Inactive drugs assessment
• Unmet Needs

Key Questions

Current Treatment Scenario and Emerging Therapies:

• How many companies are developing Idiopathic Pulmonary Fibrosis drugs?
• How many Idiopathic Pulmonary Fibrosis drugs are developed by each company?
• How many emerging drugs are in mid-stage, and late-stage of development for the treatment of Idiopathic Pulmonary Fibrosis?
• What are the key collaborations (Industry-Industry, Industry-Academia), Mergers and acquisitions, licensing activities related to the Idiopathic Pulmonary Fibrosis therapeutics?
• What are the recent trends, drug types and novel technologies developed to overcome the limitation of existing therapies?
• What are the clinical studies going on for Idiopathic Pulmonary Fibrosis and their status?
• What are the key designations that have been granted to the emerging drugs?

Key Players

• FibroGen
• United Therapeutics
• Bellerophon Therapeutics
• MediciNova
• Novartis
• Endeavor BioMedicines
• Pliant Therapeutics
• Nitto Denko
• Kadmon Pharmaceuticals
• Calliditas Therapeutics
• Avalyn Pharmaceuticals
• PureTech Health
• Taiho Pharmaceutical
• Bristol-Myers Squibb
• Galecto Biotech AB
• CSL Behring
• Celgene Pharmaceutical
• Vicore Pharma
• Boehringer Ingelheim
• Guangdong Raynovent
• Sunshine Lake Pharma co
• Suzhou Zelgen Biopharmaceuticals
• Algernon Pharmaceuticals
• Horizon Therapeutics
• Daewoong Pharmaceutical
• Metagone Biotech
• AstraZeneca
• Lung Therapeutics
• Bridge Biotherapeutics
• AstraZeneca
• Kinarus AG
• Insmed
• Reviva Pharmaceuticals
• Annapurna Bio
• Guangdong Hengrui Pharmaceutical Co., Ltd.
• Ark Biosciences
• Ocean Biomedical

Key Products

• Pamrevlumab
• Treprostinil
• Nitric oxide inhalation - INOpulse
• MN-001 (tipelukast)
• VAY736
• ENV-101
• PLN-74809
• ND-L02-s0201
• KD025
• GKT137831
• AP 01
• LYT-100
• TAS-115
• BMS-986278
• GB0139
• CSL312
• CC-90001
• C21
• BI1015550
• ZSP1603
• HEC585
• Jaktinib Dihydrochloride Monohydrate
• Ifenprodil
• HZN-825
• DWN12088
• MG-S-2525
• Saracatinib
• LTI-03
• BBT-877
• AZD5055
• KIN001-IPF
• Treprostinil palmitil inhalation powder (TPIP)
• Brilaroxazine
• ANPA 0073
• SHR 1906
• AK 3280
• OCF 203

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