Bullous pemphigoid - Pipeline Insight, 2024

This “Bullous pemphigoid- Pipeline Insight, 2024” report provides comprehensive insights about 5+ companies and 5+ pipeline drugs in Bullous pemphigoid 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

Bullous pemphigoid: Understanding

Bullous pemphigoid: Overview

Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disorder, representing 80% of subepidermal immunobullous cases. Bullous pemphigoid most commonly affects elderly patients between the ages of 60 to 80 years. While the clinical presentation of bullous pemphigoid is broad, the immunobullous skin disorder characteristically presents with tense bullae and intense generalized pruritus. In atypical cases, bullous lesions may be absent, and these cases require a high degree of clinical suspicion. A biopsy for hematoxylin and eosin staining will show a subepidermal split with eosinophils, and direct immunofluorescence will highlight the autoantibodies against the basement membrane zone. ELISA testing is also useful in diagnosing bullous pemphigoid. Treatment depends on the severity of the disease; however, standard therapies involve topical or systemic immunosuppressive agents. The prognosis varies, and long-term monitoring is often required.

There are two main components to the pathophysiology of BP: immunologic and inflammatory. The immunologic elements comprise autoantibodies against two parts of the basal keratinocyte hemidesmosomal proteins, BP antigen 230 (BPAG1) and BP antigen 180 (BPAG2 or type XVII collagen). These antigens play an essential part in the adhesion complexes that promote epithelial-stromal adhesion. When autoantibodies bind to their target antigen, the inflammatory component follows, activating complement and mast cells. This causes neutrophils and eosinophils to release various inflammatory cells, resulting in the release of proteolytic enzymes that damage the dermal-epidermal junction.

In the prodromal phase, patients may experience moderate-to-severe pruritus alone or associated with urticarial or papular lesions. This later evolves to bullae in weeks to months, typically present in the axillae, on the flexor surface of the forearms, medial thighs, trunk, and abdomen. Approximately 20% of patients will have neither bullae nor erosions at the time of presentation. Constitutional symptoms are uncommon, except in widespread, severe disease. The bullous phase is characterised by the presence of vesicles and bullae on a background of normal or erythematous skin. The blisters are tense, up to 1 to 4 centimeters in diameter, and sometimes hemorrhagic. They typically contain a clear fluid and may persist for several days before leaving erosions and crusts. Unlike pemphigus vulgaris, the Nikolsky sign is negative in typical cases of bullous pemphigoid.

Childhood cases are rare. However, there have been cases of infantile bullous pemphigoid, which presents with an acral distribution in the first year of life. The other variant of bullous pemphigoid of childhood are cases localized to the vulva. These cases need to be distinguished from other disorders due to their rarity. Therefore, direct immunofluorescence may be necessary. In some childhood cases, IgA antibodies against the NC16A domain of BP180 have been noted. It is also reported that the infancy subtype is not related to a maternofetal transfer of autoantibodies.

Researchers debate whether bullous pemphigoid is associated with internal malignancies. However, most believe this association is due to the older age of the patient. A recent study found no increased risk for concurrent or subsequent malignancies in patients with bullous pemphigoid.

Bullous pemphigoid- Pipeline Insight, 2024 report outlays comprehensive insights of present scenario and growth prospects across the indication. A detailed picture of the Bullous pemphigoid pipeline landscape is provided which includes the disease overview and Bullous pemphigoid treatment guidelines. The assessment part of the report embraces, in depth Bullous pemphigoid 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, Bullous pemphigoid 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 Bullous pemphigoid R&D. The therapies under development are focused on novel approaches to treat/improve Bullous pemphigoid.

Bullous pemphigoid Emerging Drugs Chapters

This segment of the Bullous pemphigoid 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.

Bullous pemphigoid Emerging Drugs

Dupilumab: Regeneron/Sanofi Dupilumab (REGN-668) is a fully human mAb to IL-4 receptor a (IL-4Ra) that inhibits both IL-4 and IL-13 signaling. Dupilumab (Dupixent) is approved in the U.S. to treat patients aged 6 years and older with moderate-to-severe atopic dermatitis. Dupixent is also approved in the EU and Japan to treat certain adults with severe CRSw NP. The drug is in Phase III clinical studies for Bullous Pemphigoid. Dupilumab is being jointly developed by Regeneron and Sanofi under a global collaboration agreement. Dupilumab was invented using Regeneron's proprietary VelocImmune technology. The FDA has granted orphan drug designation to dupilumab for the treatment ofBullousPemphigoid.

Bullous pemphigoid: Therapeutic Assessment

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

Major Players in Bullous pemphigoid

• There are approx. 6+ key companies which are developing the therapies for Bullous pemphigoid. The companies which have their Bullous pemphigoid drug candidates in the most advanced stage, i.e. phase III include, AstraZeneca.

Phases

This report covers around 5+ 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

Bullous pemphigoid 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.

Bullous pemphigoid: Pipeline Development Activities

The report provides insights into different therapeutic candidates in phase II, I, preclinical and discovery stage. It also analyses Bullous pemphigoid 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 Bullous pemphigoid drugs.

Bullous pemphigoid Report Insights

• Bullous pemphigoid Pipeline Analysis
• Therapeutic Assessment
• Unmet Needs
• Impact of Drugs

Bullous pemphigoid 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 Bullous pemphigoid drugs?
• How many Bullous pemphigoid drugs are developed by each company?
• How many emerging drugs are in mid-stage, and late-stage of development for the treatment of Bullous pemphigoid?
• What are the key collaborations (Industry-Industry, Industry-Academia), Mergers and acquisitions, licensing activities related to the Bullous pemphigoid 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 Bullous pemphigoid and their status?
• What are the key designations that have been granted to the emerging drugs?

Key Players

• AstraZeneca
• Sanofi
• Innate Pharma

Key Products

• Benralizumab
• Dupilumab
• Avdoralimab

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