Kiniksa Pharmaceuticals International, Plc · NASDAQ:KNSA

Kiniksa Pharmaceuticals International — Investor Presentation (October 2026)

47 slides from Kiniksa Pharmaceuticals International's investor presentation, October 2026

1. Slide 1: Corporate Presentation

   Corporate Presentation OCTOBER 2026

2. Slide 2: Forward Looking Statements

   Forward Looking Statements This presentation (together with any other statements or information that we may make in connection herewith) contains forward-looking statements with respect to Kiniksa Pharmaceuticals International, plc (and its consolidated subsidiaries, collectively, unless context oth

3. Slide 3: Addressing Unmet Need is at the Heart of What We Do

   Addressing Unmet Need is at the Heart of What We Do Building on our successful foundation by prioritizing development of novel therapies for cardiovascular indications Advancing Clinical Maintaining Strong Portfolio Financial Position Established leadership in recurrent Conducting KPL-387 Phase 2/3

4. Slide 4: Innovative Portfolio of Commercial and Clinical-Stage Assets

   Innovative Portfolio of Commercial and Clinical-Stage Assets Developing novel therapies for diseases with unmet need, prioritizing cardiovascular indications Program Indication Preclinical Phase 1 Phase 2 Phase 3 Commercial SPECIALTY CARDIOVASCULAR Recurrent Pericarditis ARCALYST® (rilonacept)1-3 IL

5. Slide 5: ARCALYST

   ARCALYST ® IL-1α AND IL-1β CYTOKINE TRAP 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 ris

6. Slide 6: Pericarditis Patient Turnover Creates Steady Opportunity for Treatment

   Pericarditis Patient Turnover Creates Steady Opportunity for Treatment Of the 14,000 annual patients with multiple recurrences, there is a high turnover of ~50% of patients each year, meaning ongoing opportunities to ensure diagnosis and targeted treatment Approximately 14,000 recurrent pericarditis

7. Slide 7: IL-1α and IL-1β in the Autoinflammatory Cycle of Recurrent Pericarditis

   IL-1α and IL-1β in the Autoinflammatory Cycle of Recurrent Pericarditis In addition to inflammatory cytokines such as IL-6, promotion and progression of the inflammatory process in pericarditis is due to IL-1α and IL-1β CRP, C-reactive protein; DAMPs, damage- associated molecular patterns; IL, inter

8. Slide 8: ARCALYST is Evolving the Treatment Landscape for Recurrent Pericarditis

   ARCALYST is Evolving the Treatment Landscape for Recurrent Pericarditis In the three years following approval, ARCALYST increasingly replaced corticosteroids after NSAIDs and colchicine RESONANCE RWE: Expert Centers in U.S. ACC Concise Clinical Guidance: First U.S. Formal Guidance Second-Line use in

9. Slide 9: Key Executional Priorities to Drive Greater Patient and Physician Adoption

   Key Executional Priorities to Drive Greater Patient and Physician Adoption % of Physicians Considering ARCALYST by Recurrence1 (n= 200 Cardiologists / Rheumatologists) Drive physician awareness of the 2025 ACC Concise 80% Clinical Guidance 69% 67% 51% 54% 40% Advance direct-to-patient digital market

10. Slide 10: Driving Penetration in Initial Target Population with Upside Opportunity

    Driving Penetration in Initial Target Population with Upside Opportunity ARCALYST Label Covers Recurrent Pericarditis Increasing Penetration into (Annual Epidemiology of Approximately 40,000) Multiple Recurrence 1st Recurrence Population ~26,000 23% A sizable portion could be Additional ~20% of pati

11. Slide 11: Strong Commercial Fundamentals Support Continued ARCALYST Growth

    Strong Commercial Fundamentals Support Continued ARCALYST Growth $286.0M >5,4501 Q3 2026 Net Revenue Total Prescribers Representing ~58% YoY Growth ~1,640 Repeat Prescribers ~3 Years1 ~23% 2 Average Total Duration of Therapy In Line with Median Penetration into Multiple Recurrence Duration of Diseas

12. Slide 12: Heart's Home DTC Campaign is Showing Strong Signals of Success

    Heart’s Home DTC Campaign is Showing Strong Signals of Success Market Research Findings1 Website Activity Improvements2 • ~20% of patients recall seeing the ARCALYST ad since its • 12x increase in weekly visits to ARCALYST.com relative to launch pre-DTC period • >50% of patients seeing the ad indica

13. Slide 13: Growth in Total Patients on ARCALYST Therapy

    Growth in Total Patients on ARCALYST Therapy Acceleration in new-to-brand and restart patients offset higher patient stops over time ARCALYST Patient Flow Illustrative Patient Flow Strong steady growth: >5,450 New to Brand Patients unique prescribers; ~30% of which are repeat prescribers Increases o

14. Slide 14: ARCALYST Enables Effective Treatment Throughout Duration of Disease

    ARCALYST Enables Effective Treatment Throughout Duration of Disease Patients with multiple recurrences often continue suffering from flares for a median of 3 years1 Average Total Duration of Therapy Natural History of Patients with Multiple Recurrences ⅓ of Patients with Multiple Recurrences Suffer

15. Slide 15: Expanding Breadth and Depth of ARCALYST Prescribing

    Expanding Breadth and Depth of ARCALYST Prescribing Total and Repeat Prescribers of ARCALYST for The Growing Repeat Prescriber Base is Delivering >50% Recurrent Pericarditis Patients of All New Patient Prescriptions 2 Patients >5,450 ~1,625 >3,825 3 Patients ~1,275 ≥4 Patients >2,550 ~1,100 Q3 2021

16. Slide 16: Pricing, Access, and Distribution Considerations

    Pricing, Access, and Distribution Considerations Pricing Access Distribution • ARCALYST list price of $25,158 per month • Kiniksa’s goal is to maintain rapid and broad • ARCALYST is distributed through a closed access to ARCALYST for patients with Recurrent network of designated specialty pharmacies

17. Slide 17: 2026 ARCALYST Net Product Sales Guidance

    2026 ARCALYST Net Product Sales Guidance Revenue guidance raised to $1.045-$1.050B from $980-$995M $1.045-1.050B Expected Net Product Sales Position ARCALYST as 2nd line Standard of Care $677.6M Deepen penetration into 2+ recurrence population $417.0M Drive adoption among larger 1st recurrence $233.

18. Slide 18: Summary of ARCALYST Profit Share Arrangement with Regeneron

    Summary of ARCALYST Profit Share Arrangement with Regeneron1 ARCALYST Net Sales (CAPS + DIRA + Recurrent Pericarditis)2 Minus 100% of Profit Split Eligible Cost of Goods Sold3 • Kiniksa is responsible for sales and distribution of ARCALYST in all approved Minus 100% of ARCALYST Field Force Expenses

19. Slide 19: KPL-387

    KPL-387 MONOCLONAL ANTIBODY IL-1 RECEPTOR ANTAGONIST INHIBITING IL-1α AND IL-1β SIGNALING DISEASE AREA: Recurrent pericarditis; painful and debilitating autoinflammatory cardiovascular disease SCIENTIFIC RATIONALE: Inhibition of IL-1α and IL-1β signaling well-established for the treatment for recurr

20. Slide 20: KPL-387: Independently Developed IL-1 Receptor Antagonist for Recurrent Pericarditis

    KPL-387: Independently Developed IL-1 Receptor Antagonist for the Treatment of Recurrent Pericarditis KPL-387 • Fully human IgG2 monoclonal antibody • Binds to IL-1R1, inhibiting both IL-1α & IL-1β cytokine-mediated signaling • IL-1α and IL-1β inhibition is well-established and well-tolerated • Mont

21. Slide 21: KPL-387 Program Aims to Address Key Patient Needs and Expand Market

    KPL-387 Program Aims to Address Key Patient Needs and Expand Market The vast majority of surveyed HCPs report that an efficacious IL-1α & IL-1β inhibitor with the target profile of KPL-387 would be best positioned to address unmet needs of patients living with recurrent pericarditis and is likely to

22. Slide 22: KPL-387 Development Program

    Registrational Program Includes Pivotal and Supplemental Components Treatment Phase Study Design & Type Patient Population Duration Event-Driven, Double-Blind, Pivotal Phase 3 Placebo-Controlled, Randomized- Qualifying Pericarditis Episode Event-Driven Study Withdrawal Study1 KPL-387 Development Pro

23. Slide 23: KPL-387 Development Program Builds on Successful RHAPSODY Design

    KPL-387 Development Program Builds on Successful RHAPSODY Design Phase 2/3 recurrent pericarditis clinical trial Population: Primary Efficacy Endpoint • Up to ~80 patients diagnosed with RP • Time to Treatment Response2 presenting at screening with a qualifying Key Secondary Endpoints pericarditis e

24. Slide 24: Dose-Focusing Portion Builds on Precedent of Rilonacept Phase 2 Trial

    Dose-Focusing Portion Builds on Precedent of Rilonacept Phase 2 Trial Cadence & magnitude of initial response and duration of action in Phase 2 inform Phase 3 efficacy, recurrence risk reduction Phase 2 Dose-Focusing Portion of Open-Label Phase 2 Clinical Trial of Rilonacept in Pericarditis Populati

25. Slide 25: Phase 2 Data Supported Selection of KPL-387 300 mg SC q4wk Dose Level

    Phase 2 Data Supported Selection of KPL-387 300 mg SC q4wk Dose Level for Pivotal Phase 3 Study Primary and Secondary Endpoints* KPL-387 300 mg SC q4wk Time to Treatment Response1 4.0 (3.0, 6.0) days Time to Pain Response 4.0 (3.0, 6.0) days Time to CRP Normalization2 8.0 (7.0, 9.0) days *Reported a

26. Slide 26: Financials Third Quarter 2026

    Financials Third Quarter 2026

27. Slide 27: Third Quarter 2026 Financial Results

    Third Quarter 2026 Financial Results Three Months Ended September 30, Income Statement Three Months Ended September 30, Collaboration Expenses1 2026 2025 2026 2025 ARCALYST Net Sales $286.0M $180.9M Product Revenue $286.0M $180.9M Profit Split-Eligible Cost of Goods Sold ($26.2M) ($20.0M) License an

28. Slide 28: Appendix: Out-Licensing Agreements

    Appendix Out-Licensing Agreements

29. Slide 29: Out-Licensing Agreements

    Out-Licensing Agreements Partnership with Huadong Medicine Gives Kiniksa Opportunity to Expand Footprint into Asia Pacific Region (Excluding Japan) • In February 2022, Kiniksa announced a strategic collaboration with Huadong to develop and commercialize ARCALYST in Greater China and multiple other c

30. Slide 30: Appendix: ARCALYST

    Appendix ARCALYST (rilonacept)

31. Slide 31: ARCALYST Monotherapy Provided Long-Term Prevention of Recurrence

    ARCALYST Monotherapy Provided Long-Term Prevention of Recurrence Over Three-Year Duration of RHAPSODY RISK OF PERICARDITIS RECURRENCE WHILE ON ARCALYST MONOTHERAPY*1 FREEDOM FROM RECURRENCE (%) ** TIME ON CONTINUOUS ARCALYST MONOTHERAPY (MONTHS) A new integrated analysis of RHAPSODY supports evidenc

32. Slide 32: Real-World Outcomes Data Affirm Benefit of Sustained ARCALYST Treatment

    Real-World Outcomes Data Affirm Benefit of Sustained ARCALYST Treatment Rilonacept provides superior clinical outcomes and reduced hospitalizations/ED visits versus 2nd-line glucocorticoids Clinical Outcomes and Healthcare Resource Utilization in Patients With RP *Patients remaining on NSAIDs ± Colc

33. Slide 33: RHAPSODY Design

    RHAPSODY Design Event-Driven Pivotal Study Long-Term Extension (LTE) (up to 24 months) Median rilonacept treatment duration prior to the LTE (RI+RW) was 9 months (range, 3-14) End of Treatment Primary Efficacy Endpoint 18 months after the most End of Study Loading Dose Randomization Time-to-First-Ad

34. Slide 34: Rilonacept Initiation Resulted in Rapid Resolution of Acute Pericarditis Episodes

    Rilonacept Initiation Resulted in Rapid Resolution of Acute Pericarditis Episodes RHAPSODY run-in period 6 6 Most patients experienced treatment response*, pain relief, and inflammation resolution as 97 % of patients (77 of 79) taking early as after the first dose2 ARCALYST experienced 4 treatment r

35. Slide 35: 96% Reduction in Risk of Pericarditis Recurrence

    96% Reduction in Risk of Pericarditis Recurrence Pivotal Phase 3 RHAPSODY Data ARCALYST reduced the risk of pericarditis recurrence The primary efficacy endpoint was time to first adjudicated pericarditis recurrence in the randomized withdrawal period. The median time to recurrence on ARCALYST could

36. Slide 36: RHAPSODY Long-Term Extension Data

    RHAPSODY Long-Term Extension Data Demonstrated Rilonacept Treatment Beyond 18 Months Resulted in Continued Treatment Response1 ARCALYST reduced the risk of pericarditis recurrence Recurrence-free rate was 97% (32 of 33) for ARCALYST and 25% (2 of 8) for those who suspended therapy 3% (1 of 33) of pa

37. Slide 37: Randomized Phase 2 Trial of Rilonacept in Cardiac Sarcoidosis

    Randomized Phase 2 Trial of Rilonacept in Cardiac Sarcoidosis Collaborative study agreement with Mayo Clinic and The Johns Hopkins University PROBE-design study to evaluate efficacy and safety of rilonacept over 24 weeks of treatment in participants with cardiac sarcoidosis1 D1/Week 0 Week 12 Week 2

38. Slide 38: Appendix: KPL-387

    © Appendix KPL-387

39. Slide 39: KPL-387 Phase 2/3 Recurrent Pericarditis Clinical Trial: Phase 2

    KPL-387 Phase 2/3 Recurrent Pericarditis Clinical Trial Phase 2: Dose-focusing study DOSE-FOCUSING : TREATMENT PERIOD (WEEKS 0-24) KPL-387 300 mg SC q2wk KPL-387 300 mg SC q4wk R LONG-TERM EXTENSION Screening 1:1:1:1 (UP TO 24 MONTHS) 2 KPL-387 100 mg SC q2wk KPL-387 100 mg SC q4wk NSAIDs and/or col

40. Slide 40: KPL-387 Phase 2/3 Recurrent Pericarditis Clinical Trial: Phase 3

    KPL-387 Phase 2/3 Recurrent Pericarditis Clinical Trial Phase 3: Pivotal study R Primary Efficacy Endpoint 1:1 Time-to-first-adjudicated Pericarditis Recurrence3 Screening Single-Blind Run-In Period1 Event-Driven, Double-Blind, Placebo-Controlled, Long-Term Extension Period Randomized-Withdrawal Per

41. Slide 41: Transition to KPL-387 Monotherapy Dosing & Administration Study

    Transition to KPL-387 Monotherapy Dosing & Administration Study Supplemental Phase 2 study evaluating the transition of patients from standard therapies to KPL-387 monotherapy Phase 2 Transition to KPL-387 Monotherapy Dosing & Administration Long-Term Extension (Weeks 0-16) (Up to 24 months3) Prior

42. Slide 42: KPL-387 Phase 1 SAD/MAD Study

    KPL-387 Phase 1 SAD/MAD Study First-in-human study evaluating safety, tolerability, pharmacokinetics, and immunogenicity Part A: Single Ascending Dose Cohorts (1-11) Part B: Multiple Ascending Dose Cohorts (12-14) SC Administration IV Administration SC Administration 11 1000 mg (6 active/2 placebo)

43. Slide 43: The Single-Dose PK of KPL-387 Support the Monthly Dose Paradigm

    The Single-Dose PK of KPL-387 Support the Monthly Dose Paradigm Topline data from KPL-387 Phase 1 single ascending dose study KPL-387 Pharmacokinetics (Subcutaneous Administration) in Healthy Volunteers PK = Pharmacokinetics 43

44. Slide 44: PK-Modeling and Dose Simulations for KPL-387 Phase 2/3 RP Study

    PK-Modeling and Dose Simulations for KPL-387 Phase 2/3 RP Study Predicted KPL-387 Serum Concentration (μg/mL) Target Concentration Pharmacokinetic modeling of KPL-387 at the anticipated therapeutic dose supports the monthly dose paradigm 44

45. Slide 45: Appendix: KPL-1161

    , a KINIKSA XZ Appendix KPL-1161

46. Slide 46: IL-1α and IL-1β Inhibition is Well Established in Cardiovascular and Inflammatory Diseases

    IL-1α and IL-1β Inhibition is Well Established in Cardiovascular and Inflammatory Diseases DIRA Indications with sJIA & Still’s Disease Regulatory Approval Periodic Fever Syndrome± Recurrent Pericarditis or Positive RCT Hidradenitis Suppurativa Trials Rheumatoid Arthritis* Acute Gout Flares* ASCVD*

47. Slide 47: Corporate Presentation

    Corporate Presentation OCTOBER 2026

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