Primary Biliary Cholangitis - Pipeline Insight, 2026

DelveInsight’s, “Primary Biliary Cholangitis - Pipeline Insight, 2026” report provides comprehensive insights about 20+ companies and 25+ pipeline drugs in Primary Biliary Cholangitis 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

Primary Biliary Cholangitis: Understanding

Primary Biliary Cholangitis: Overview

Primary biliary cholangitis (PBC) is a chronic, progressive autoimmune liver disease in which the body’s immune system gradually damages and destroys the small bile ducts inside the liver (intrahepatic bile ducts). This leads to impaired bile flow (cholestasis), causing bile to accumulate in the liver, which results in inflammation, fibrosis, and eventually cirrhosis and liver failure if untreated. It is classified as an autoimmune cholestatic liver disease and is more common in middle-aged women.

From a clinical perspective, PBC often presents insidiously. Many patients are asymptomatic at diagnosis, but the most common early symptoms include fatigue and pruritus (itchy skin). As the disease progresses, patients may develop right upper abdominal discomfort, dry eyes and mouth (associated autoimmune conditions), skin hyperpigmentation, xanthomas, jaundice, edema, and weight loss due to advancing liver dysfunction.

The exact cause of PBC is not fully understood, but it is widely considered an autoimmune disorder in which immune-mediated destruction of bile duct cells occurs. Evidence suggests a combination of genetic predisposition and environmental triggers (such as infections, toxins, or chemicals) initiates the disease process. This immune response leads to chronic inflammation and progressive bile duct injury.

Regarding treatment, there is no definitive cure, but therapies aim to slow disease progression and manage symptoms. The first-line treatment is ursodeoxycholic acid (UDCA), which improves bile flow and delays progression. In patients with inadequate response, obeticholic acid may be added. Symptomatic treatments (e.g., for itching, fatigue) and management of complications are important, and in advanced cases with liver failure, liver transplantation may be required.

"" Primary Biliary Cholangitis - Pipeline Insight, 2026"" report by DelveInsight outlays comprehensive insights of present scenario and growth prospects across the indication. A detailed picture of the Primary Biliary Cholangitis pipeline landscape is provided which includes the disease overview and Primary Biliary Cholangitis treatment guidelines. The assessment part of the report embraces, in depth Primary Biliary Cholangitis 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, Primary Biliary Cholangitis 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 Primary Biliary Cholangitis R&D. The therapies under development are focused on novel approaches to treat/improve Frontotemporal Dementia.

Primary Biliary Cholangitis Emerging Drugs Chapters

This segment of the Primary Biliary Cholangitis 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.

Primary Biliary Cholangitis Emerging Drugs

• Saroglitazar: Zydus Therapeutics Inc.

Saroglitazar is an investigational, orally administered small-molecule drug developed by Zydus Therapeutics Inc., characterized as a dual peroxisome proliferator-activated receptor (PPAR) α/γ agonist. This mechanism enables it to regulate lipid metabolism, inflammation, and bile acid homeostasis, making it particularly relevant for chronic liver diseases. The drug is being developed for primary biliary cholangitis (PBC), especially in patients who have an inadequate response or intolerance to ursodeoxycholic acid, and is also under investigation for non-alcoholic steatohepatitis (NASH). Saroglitazar has demonstrated promising safety and efficacy in clinical studies and has advanced into a Phase III (late-stage) clinical trial (EPICS-III) for PBC, with positive topline results reported. Additionally, the drug has received important regulatory support, including Fast Track Designation and Orphan Drug Designation from the US FDA, highlighting its potential to address an unmet medical need in this rare liver disease. Saroglitazar is currently a late-stage (Phase III) pipeline drug for PBC.

• CNP‑104: COUR Pharmaceutical Development Company, Inc.

CNP‑104 is an investigational, biodegradable nanoparticle therapy being developed by COUR Pharmaceutical Development Company, Inc. for Primary Biliary Cholangitis (PBC). The drug uses a proprietary antigen‑specific immune tolerance platform designed to modulate the immune system at the root cause of PBC by encapsulating the E2 component of the mitochondrial pyruvate dehydrogenase complex (PDC‑E2), a key autoantigen in PBC. This targeted mechanism aims to reprogram pathogenic CD4+ and CD8+ T‑cells, halt bile duct destruction, and potentially slow or stop disease progression rather than merely treating symptoms. CNP‑104 has completed early clinical development and shown positive results from a Phase 2a clinical trial in PBC patients, demonstrating favorable immune responses and reductions in liver stiffness, a surrogate indicator of fibrosis and disease progression. COUR has received two regulatory designations from the U.S. Food and Drug Administration (FDA) for CNP‑104 in PBC,Fast Track Designation (granted January 2022) and Orphan Drug Designation (granted December 2024), reflecting its potential to address an unmet need in this rare autoimmune liver disorder and enabling regulatory incentives such as priority review and market exclusivity.

• Pemafibrate: Kowa Company, Ltd.

Pemafibrate is an orally administered, selective peroxisome proliferator‑activated receptor‑alpha (PPARα) modulator (SPPARMα) developed by Kowa Research Institute, Inc. and its parent Kowa Company, Ltd. as an advanced fibrate class molecule designed to regulate lipid metabolism by activating the PPARα nuclear receptor, leading to enhanced fatty acid oxidation, reduced triglyceride levels, and improved high‑density lipoprotein (HDL) cholesterol profiles with a superior benefit‑risk balance compared with conventional fibrates in dyslipidemia. Pemafibrate is also in Phase II clinical development for the indication of Primary Biliary Cholangitis (PBC), reflecting an expanded liver disease profile, and it has received Orphan Drug Designation from the US FDA for the treatment of PBC.

Further product details are provided in the report……..

Primary Biliary Cholangitis: Therapeutic Assessment

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

• Major Players in Primary Biliary Cholangitis

There are approx. 20+ key companies which are developing the therapies Primary Biliary Cholangitis. The companies which have their Primary Biliary Cholangitis drug candidates in the most advanced stage, i.e. Phase III include, Zydus Therapeutics, and others.

• Phases

DelveInsight’s report covers around 25+ 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

Primary Biliary Cholangitis pipeline report provides the therapeutic assessment of the pipeline drugs by the Route of Administration. Products have been categorized under various ROAs such as

• Intra-articular
• Intraocular
• Intrathecal
• Intravenous
• Ophthalmic
• Oral
• Parenteral
• Subcutaneous
• Topical
• Transdermal
• Molecule Type

Products have been categorized under various Molecule types such as

• Oligonucleotide
• Peptide
• Small molecule
• Product Type

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

Primary Biliary Cholangitis: Pipeline Development Activities

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

Primary Biliary Cholangitis Report Insights

• Primary Biliary Cholangitis Pipeline Analysis
• Therapeutic Assessment
• Unmet Needs
• Impact of Drugs

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

Key Players

• Zydus Therapeutics Inc.
• COUR Pharmaceutical Development Company, Inc
• Kowa Research Institute, Inc.
• Parvus Therapeutics, Inc.
• Calliditas Therapeutics Suisse SA
• HighTide Biopharma Pty Ltd
• Mirum Pharmaceuticals, Inc.
• Hepagene

Key Products

• Saroglitazar
• CNP-104
• Pemafibrate
• PVT201
• Setanaxib
• HTD1801
• Volixibat
• HPG1860

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