Kiniksa Pharmaceuticals International : Investor Presentation – April 2025

KNSA

Corporate Presentation

Building on our successful foundation by prioritizing development of novel therapies for cardiovascular indications

Current operating plan expected to remain cash flow positive on an annual basis

Established leadership in recurrent

pericarditis market

Continued growth potential with

only ~13% penetration into target population1

Expected 2025 ARCALYST revenue

of $590-$605M

Advancing Clinical

Portfolio

Developing KPL-387 in recurrent

pericarditis

KPL-387 Phase 2/3 trial to initiate in

mid-2025; Phase 2 data expected in

2H 2026

IND-enabling activities with

KPL-1161

Maintaining Strong

Financial Position

Q1 2025 cash reserves of ~$268M

Thoughtful capital allocation

Financial strength provides capacity

to continue investing in additional

value creation

1) As of year-end 2024.

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Developing novel therapies for diseases with unmet need, prioritizing cardiovascular indications

Program

Indication

Preclinical

Phase 1

Phase 2

Phase 3

Commercial

SPECIALTY CARDIOVASCULAR

ARCALYST® (rilonacept)1-3

IL-1α & IL-1β Trap

Recurrent Pericarditis

Cardiac Sarcoidosis

Collaborative Study Agreement with Mayo Clinic & The Johns Hopkins University

KPL-387

IL-1 Antagonist mAb

Recurrent Pericarditis

KPL-1161

Fc-Modified IL-1 Antagonist mAb

Undisclosed

OTHER (NON-CARDIOVASCULAR)

Abiprubart

Anti-CD40 mAb

Exploring Strategic Alternatives

Program

Licensee

Exclusive Licensed Territory

OUT-LICENSING AGREEMENTS

ARCALYST (rilonacept)

IL-1α & IL-1β Trap

Huadong Medicine

Asia Pacific Region, Excluding Japan

Vixarelimab

Anti-OSMRβ mAb

Roche and Genentech

Worldwide

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1) Approved in the U.S.; ARCALYST is also approved in the U.S. for cryopyrin-associated periodic syndromes (CAPS) and deficiency of the interleukin-1 receptor antagonist (DIRA); 2) The FDA granted Breakthrough Therapy designation to ARCALYST for recurrent pericarditis in 2019; the FDA granted Orphan Drug exclusivity to ARCALYST in March 2021 for the treatment of recurrent pericarditis and reduction in risk of recurrence in adults and pediatric patients 12 years and older. The European Commission granted Orphan Drug designation to ARCALYST for the treatment of idiopathic pericarditis in 2021; 3) Kiniksa has worldwide rights, excluding the Middle East and North Africa; Kiniksa granted Huadong Medicine exclusive rights in the Asia Pacific Region, excluding Japan.

IL-1α = interleukin-1α; IL-1β = interleukin-1β; IL-1 = interleukin-1; mAb = monoclonal antibody; OSMRβ = oncostatin M receptor beta

ARCALYST ®

DISEASE AREA: Recurrent pericarditis1; painful and debilitating autoinflammatory cardiovascular disease

COMPETITION2: First and only FDA-approved therapy for recurrent pericarditis

REGULATORY: U.S. Orphan Drug exclusivity for treatment of and reduction in risk of recurrence of recurrent pericarditis; European Commission Orphan Drug designation in idiopathic pericarditis

STATUS: FDA-Approved

ECONOMICS: 50/50 split on profit and third-party proceeds

RIGHTS: Kiniksa has worldwide rights3 (excluding MENA) for all indications outside those in oncology and local administration to the eye or ear

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1) ARCALYST is also approved and marketed for Cryopyrin-Associated Periodic Syndromes (CAPS) and maintenance of remission of Deficiency of Interleukin-1 Receptor Antagonist (DIRA) in the United States; 2) Drugs@FDA: ARCALYST Prescribing Information, Ilaris Prescribing Information, Kineret Prescribing Information; Kaiser et al. Rheumatol Int (2012) 32:295-299; Theodoropoulou et al. Pediatric Rheumatology 2015, 13(Suppl 1):P155; Fleischmann et al, 2017 ACR/ARHP Abstract 1196; Kosloski et al, J of Clin Pharm 2016, 56 (12) 1582-1590; Cohen et al. Arthritis Research & Therapy 2011, 13:R125; Cardiel et al. Arthritis Research & Therapy 2010, 12:R192; Hong et al. Lancet Oncol 2014, 15: 656-666; 3) Kiniksa granted Huadong Medicine exclusive rights in the Asia Pacific Region, excluding Japan.

IL-1α = interleukin-1α ; IL-1β = interleukin-1β; MENA = Middle East North Africa

Disclaimer

Kiniksa Pharmaceuticals International plc published this content on April 29, 2025, and is solely responsible for the information contained herein. Distributed via Public Technologies (PUBT), unedited and unaltered, on April 29, 2025 at 13:04 UTC.