AB2 Bio

What AB2 Bio does

AB2 Bio Ltd. is a clinical-stage biopharmaceutical company founded in 2010 and headquartered at the EPFL Innovation Park in Lausanne, Switzerland. The company is developing Tadekinig alfa, a recombinant human Interleukin-18 Binding Protein (r-hIL-18BP) that binds free IL-18 to restore the IL-18/IL-18BP balance and reduce pathological inflammation in patients with IL-18 driven hyperinflammatory diseases. The lead indication is Primary Monogenic Hemophagocytic Lymphohistiocytosis (HLH) caused by NLRC4 mutation and XIAP deficiency — an ultra-rare, life-threatening, primarily pediatric condition with no approved therapies — with additional development targeting Macrophage Activation Syndrome (secondary HLH) and Adult onset Still's Disease. AB2 Bio also owns a proprietary free IL-18 immunoassay that can serve as a companion diagnostic to identify patients most likely to respond to Tadekinig alfa.

Tadekinig alfa has received FDA Breakthrough Therapy Designation and FDA Orphan Drug Designation (2017), as well as EMA Orphan Drug Designation (2016). The pivotal Phase 3 trial in primary monogenic IL-18 driven HLH completed enrollment in March 2023. In January 2025, the company signed a U.S. option and licensing agreement with Nippon Shinyaku covering Tadekinig alfa in this indication, entitling AB2 Bio to up to USD 36 million in early payments, up to USD 150 million in development milestones, and up to USD 500 million in commercial milestones and royalties, with WuXi Biologics serving as the commercial-scale manufacturing partner.

AB2 Bio operates on a licensing/royalty revenue model and is pre-revenue; the company has raised over CHF 70 million across four financing rounds from investors in Switzerland, the EU, and the U.S. It employs between 11 and 50 people and is led by CEO Dr. Djordje Filipovic, a former Novartis commercial executive, with co-founder Avinoam Kadouri as CTO. The addressable U.S. markets for the two lead indications are reported at USD 110 million (MAS, 2024) and USD 140 million (HLH, 2024), with projected 24%+ CAGRs through 2034.

Name

AB2 Bio

Legal name

AB2 Bio Ltd.

Website

https://ab2bio.com

Company type

Private

Founded year

2010

Operating status

Operating

Headcount range

11–50 employees

Short description

AB2 Bio is a Swiss clinical-stage biopharmaceutical company developing Tadekinig alfa, a recombinant IL-18 binding protein therapy, for ultra-rare hyperinflammatory diseases including primary monogenic HLH, Macrophage Activation Syndrome, and Adult onset Still's Disease, with a proprietary free IL-18 companion diagnostic assay.

Ownership category

akta.pro rank

Product category

Biopharmaceuticals / Rare Disease Therapeutics

NAICS

Biological Product (except Diagnostic) Manufacturing (325414), Pharmaceutical Preparation Manufacturing (325412), In-Vitro Diagnostic Substance Manufacturing (325413), Research and Development in Biotechnology (except Nanobiotechnology) (541714)

SIC

Biological Products, (No Disgnostic Substances) (2836), Pharmaceutical Preparations (2834), In Vitro & In Vivo Diagnostic Substances (2835)

akta.pro primary industry

Rare Immunology, Autoimmune & Autoinflammatory Therapies (HLAIAIAE)

akta.pro secondary industries

Immunology & Inflammation Pharmaceuticals (HLAIAAAD), Immunology & Autoimmune Specialty Pharmaceuticals (HLAIACAB), Monoclonal Antibody Therapeutics (mAbs) (HLAAAAAA)

Keywords

• Rare disease therapeutics
• IL-18 binding protein
• Inflammatory disease biopharmaceuticals
• Clinical stage biotechnology
• Companion diagnostic assays

Headquarters

HQ city

Lausanne

HQ country

Switzerland

HQ region

Europe

Offices1 record

Markets served

GTM type

B2B

Offering type

Hardware or Manufacturing

Cost components

Technology or R&D, Personnel, Supply Chain, Operations, Others

Revenue model

1. Product Commercialization: AB2 Bio is developing Tadekinig alfa toward market launch. The company has a licensing agreement with Nippon Shinyaku providing upfront payments of USD 36 million, development milestones up to USD 150 million, and commercial/royalty payments up to USD 500 million.

Go-to-market motion1 record

Distribution channels2 records

Marketing channels4 records

Core offering

AB2 Bio is developing Tadekinig alfa, a recombinant human Interleukin-18 Binding Protein (r-hIL-18BP) drug candidate for the treatment of IL-18-driven inflammatory and autoimmune diseases, with primary programs in primary monogenic Hemophagocytic Lymphohistiocytosis (NLRC4 mutation and XIAP deficiency), Macrophage Activation Syndrome, and Adult onset Still's Disease. The company also provides a proprietary free IL-18 diagnostic assay that identifies patients with elevated free IL-18 and serves as a companion diagnostic to Tadekinig alfa. The product is in pivotal Phase 3 clinical trials and is being commercialized via a U.S. licensing agreement with Nippon Shinyaku.

Product overview

AB2 Bio is a drug discovery and development company with two main products: Tadekinig alfa, a recombinant Interleukin-18 Binding Protein (r-hIL-18BP) drug candidate, and a proprietary assay for detecting free IL-18. Tadekinig alfa is currently in pivotal Phase 3 clinical trials for treatment of IL-18 driven monogenic Hyperinflammatory Syndromes including NLRC4 mutation and XIAP deficiency (primary HLH), with potential applications in Macrophage Activation Syndrome and Adult onset Still's Disease. The proprietary assay serves as both a diagnostic tool and companion diagnostic to select patients most likely to respond to Tadekinig alfa treatment.

Differentiator

Problem solved

Functional benefit

Products and services

• Tadekinig alfa (recombinant human IL-18 Binding Protein) A recombinant human Interleukin-18 Binding Protein (r-hIL-18BP) with high affinity for IL-18, designed to restore the IL-18/IL-18BP balance by binding free IL-18 and thereby reduce pathological inflammation. Developed for patients with IL-18-driven inflammatory and autoimmune diseases, including primary monogenic Hemophagocytic Lymphohistiocytosis (NLRC4 mutation and XIAP deficiency), Macrophage Activation Syndrome, and Adult onset Still's Disease. Demonstrated high tolerance and excellent safety profile in Phase I, Ib, and II clinical trials; in pivotal Phase 3 trials.
• Proprietary free IL-18 diagnostic assay

Segments3 records

Ideal customer profiles3 records

Technology focussed Yes

API detail

Has API

No

API docs

API detail

Core technology

AI maturity

App detail

Feature2 records

Partnerships

Two partnerships are on record, tiered core.

• Nippon ShinyakucoreOEM/ Whitelabel/ Licensing Partner · 27 January 2025AB2 Bio signed a U.S. option and licensing agreement with Nippon Shinyaku for Tadekinig alfa. The agreement grants Nippon Shinyaku an option to acquire exclusive U.S. rights to commercialize Tadekinig alfa to treat Primary Monogenic IL-18 driven Hyperinflammatory Syndrome in patients with NLRC4 mutation and XIAP deficiency. AB2 Bio receives early payments of up to USD 36 million (including USD 6 million initial payment), with potential development milestones up to USD 150 million and commercial/royalty payments up to USD 500 million.
• WuXi BiologicscoreStrategic or Co-development Partner · 29 October 2020AB2 Bio and WuXi Biologics announced collaboration to accelerate commercial-scale manufacturing of Tadekinig alfa. WuXi Biologics to undertake commercial scale manufacturing to support U.S. market launch of the novel IL-18 binding protein therapy for orphan disease IL-18 driven monogenic Hemophagocytic Lymphohistiocytosis.

Scale indicators3 records

Recent moves6 records

Expansion highlights6 records

Direct peers

• Sobi (Swedish Orphan Biovitrum): Sobi is the most direct comparable: it developed and commercializes emapalumab (Gamifant), the only approved therapy specifically indicated for primary hemophagocytic lymphohistiocytosis (HLH) — the same disease space AB2 Bio targets with Tadekinig alfa. Both are rare-disease specialty biologics companies serving pediatric hyperinflammatory patients.
• Alexion (AstraZeneca Rare Disease): Alexion is a rare-disease biologics franchise within AstraZeneca focused on complement-mediated and severe inflammatory conditions; its orphan-drug business model, ultra-rare patient identification, and global commercial reach in pediatric specialty settings closely mirror AB2 Bio's planned Tadekinig alfa go-to-market approach.

Broad incumbents

• Novartis: Novartis markets canakinumab (Ilaris), an IL-1β inhibitor approved for Adult-onset Still's Disease, systemic Juvenile Idiopathic Arthritis, and other autoinflammatory conditions overlapping with AB2 Bio's MAS/AOSD indications, and is also headquartered in Switzerland with deep specialty immunology commercial infrastructure relevant to Tadekinig alfa's launch dynamics.
• Eli Lilly: Eli Lilly's immunology franchise includes baricitinib (Olumiant) and olokizumab, with JAK/IL-17 pathway inhibitors that are being evaluated or used off-label in HLH and other hyperinflammatory syndromes overlapping AB2 Bio's indication space, making it a meaningful large-cap comparator for specialty inflammation commercial economics.
• AbbVie: AbbVie is a major immunology player (Humira, Skyrizi, Rinvoq) with established specialty sales infrastructure in rheumatology that overlaps AB2 Bio's MAS/AOSD target specialties; its IL-23 and JAK1 inhibitors compete in the broader autoimmune inflammation landscape relevant to Tadekinig alfa.
• Pfizer: Pfizer has a broad immunology portfolio and global biologics manufacturing footprint, making it relevant both as a competitive incumbent in autoimmune inflammation and as a strategic-pharma model for how a large player might in-license an ultra-rare biologic like Tadekinig alfa.
• Roche: Roche markets tocilizumab (Actemra), an IL-6 receptor antagonist used in Adult-onset Still's Disease and other hyperinflammatory conditions that overlap AB2 Bio's target indications; Roche's deep rheumatology commercial infrastructure and companion-diagnostic capabilities are also directly comparable.
• Sanofi: Sanofi operates a major specialty immunology franchise (Dupixent, Kevzara, Sarclisa) with global rare-disease and inflammation commercial reach; its established relationships with rheumatologists and rare-disease payers provide a strategic benchmark for the type of commercial scale AB2 Bio is partnering with Nippon Shinyaku to access.

Emerging players

• Incyte: Incyte develops ruxolitinib (Jakafi), a JAK1/2 inhibitor widely used off-label for HLH and graft-versus-host disease — directly intersecting the autoinflammatory patient population Tadekinig alfa targets — and represents a competing pathway approach (JAK inhibition vs. IL-18 sequestration) in HLH/MAS.

Others

• Takeda Pharmaceutical: Takeda operates a large rare-disease and immunology franchise with relevant autoinflammatory and hematology/oncology assets, and is a relevant comparator for the orphan-disease biologics commercial playbook AB2 Bio is pursuing through the Nippon Shinyaku US partnership.

Market position

Strengths5 records

Weaknesses5 records

Competitive moat4 records

Key risks6 records

Key highlights7 records

Customer concentration

• LinkedInlinkedin.com/company/ab2-bio-ltd.

Compliance3 records

Revenue estimate

Valuation estimate

Number of profiles

Profiles6 records

Funding overview

Funding rounds1 record

Investors

Funding detail is available on the Subscription and Enterprise plan.Contact sales →

M&A

Investments

M&A and investment is available on the Subscription and Enterprise plan.Contact sales →

Frequently asked questions about AB2 Bio