Kiniksa Pharmaceuticals International, Plc · NASDAQ:KNSA

Kiniksa Pharmaceuticals International — Investor Presentation (July 2023)

38 slides from Kiniksa Pharmaceuticals International's investor presentation, July 2023

1. Slide 1: Corporate Presentation

   Corporate Presentation JULY 2023

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 within the meaning of the Private Securities Litigation Reform Act of 1995 with respect to Kiniksa Pharmaceuticals, Ltd. (and i

3. Slide 3: Portfolio of Immune-Modulating Assets

   Portfolio of Immune-Modulating Assets Program Target Preclinical Phase 1 Phase 2 Phase 3 Commercial CARDIOVASCULAR FRANCHISE ARCALYST® (rilonacept)1,2 Recurrent Pericarditis IL-1α & IL-1β Mavrilimumab3 Evaluating Development in GM-CSFRα Rare Cardiovascular Diseases AUTOIMMUNE FRANCHISE KPL-404 Rheum

4. Slide 4: Building Blocks for Value Creation in 2023 and Beyond

   Building Blocks for Value Creation in 2023 and Beyond Commercial Asset Pipeline Delivering Delivering Strong Growth for the Future Today • Expected ARCALYST net product • KPL-404 is a potentially best-in- revenue of $220-$230M in 2023, class asset; now in Phase 2 study representing ~84% growth at th

5. Slide 5: Track Record of Execution Positions Kiniksa for Continued Success

   Track Record of Execution Positions Kiniksa for Continued Success Kiniksa Continues to Utilize Business Development Expertise to Create Value • Acquired four clinical programs with differentiated mechanisms through innovative transactions and advanced to mid-/late-stage clinical trials • Executed st

6. Slide 6: 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 and mavrilimumab in Greater China, So

7. Slide 7: ARCALYST

   ARCALYST ® IL-1α AND IL-1β CYTOKINE TRAP DISEASE AREA: Recurrent pericarditis1; painful and debilitating auto-inflammatory cardiovascular disease COMPETITION2: First and only FDA-approved therapy for recurrent pericarditis REGULATORY: U.S. Orphan Drug exclusivity for treatment of and reduction in ri

8. Slide 8: Pericarditis Epidemiology

   Pericarditis Epidemiology Approximately 14,000 recurrent pericarditis patients in the U.S. suffer from persistent underlying disease, with multiple recurrences and inadequate response to conventional therapy1 ~ 160,000: Epidemiological analysis using large national surveillance databases to calculat

9. Slide 9: Early Treated Patients Are Closely Associated to the Launch Target Population

   Early Treated Patients Are Closely Associated to the Launch Target Population, While Prescribers Can Utilize ARCALYST Earlier in the Disease ARCALYST PATIENTS BY PRIOR PRODUCT 1 Colchicine Recurrent Pericarditis Annual Epidemiology: ~40,000 ~40% NSAIDS and/or Colchicine 1 ST RECURRENCE ~26,000 LOWES

10. Slide 10: Addressable U.S. Opportunity of ARCALYST Estimated to be ~14K Patients

    Addressable U.S. Opportunity of ARCALYST Estimated to be ~14K Patients ~7K new patients with multiple recurrences enter target pool annually Year -4 -3 -2 -1 0 Incident case of acute pericarditis (1st episode)1 117K 117K 117K 117K 117K Incidence of initial RP patients (1st Annual pericarditis 26K 26

11. Slide 11: Strong Q2 2023 Revenue Growth Driven by Robust Commercial Execution

    Strong Q2 2023 Revenue Growth Driven by Robust Commercial Execution Total Net Revenue Growth per Quarter $54.5M Total Prescribers >1,250 ~100% year-over-year growth Repeat Prescribers ~23% (% of Total) $27.0M Payer Approval >90% (% of Completed Cases) ~250% year-over-year growth Average Total Durati

12. Slide 12: Average Total Duration of ARCALYST Therapy Increased to ~20 Months

    Average Total Duration of ARCALYST Therapy Increased to ~20 Months1 Advancing the treatment paradigm to treat continuously throughout disease duration (median 3 years2) Average Initial ~45% Duration of Therapy Of Patients ~14 Months1 Restarted Therapy Following Initial Discontinuation Median (Within

13. Slide 13: Expanding Breadth & Depth of ARCALYST Use for Recurrent Pericarditis

    Expanding Breadth & Depth of ARCALYST Use for Recurrent Pericarditis Expanding Base of New Prescribers Pericardial Salesforce Establishing Excellence Disease Education Disease Networks ARCALYST as the Standard of Care for Recurrent Evidence Kiniksa OneConnect™ Brand Awareness Pericarditis Generation

14. Slide 14: Growth in Total Patients on Therapy Driven by Continued Acceleration in New to Brand

    Growth in Total Patients on Therapy Driven by Continued Acceleration in New to Brand and Restart Patients, Accounting for Increasing Patient Stops Over Time ARCALYST Patient Flow Illustrative Patient Flow Strong sequential growth: New to Brand Patients >1,250 unique prescribers; ~23% of which are re

15. Slide 15: 2023 ARCALYST Net Product Sales Guidance Increased

    2023 ARCALYST Net Product Sales Guidance Increased Higher expectation driven by increased prescriber adoption, patient enrollment, and duration of therapy $220-230M ARCALYST 2023 ~84% year-over-year Expected Net Product Sales growth at the midpoint $122.5M $38.5M 9 Months of Commercial Availability

16. Slide 16: Pricing, Access and Distribution Considerations

    Pricing, Access and Distribution Considerations Pricing Access Distribution • ARCALYST list price of $21,425 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: 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 Field Force Expenses indicatio

18. Slide 18: KPL-404

    KPL-404 MONOCLONAL ANTIBODY INHIBITOR INTERACTION BETWEEN CD40 AND CD154 DISEASE AREA: Rheumatoid Arthritis; a chronic inflammatory disorder affecting many joints; External proof-of-concept previously established in broad range of autoimmune diseases: Sjogren’s disease, systemic lupus, solid organ t

19. Slide 19: CD40/CD154 is an Essential Immune Pathway for T-Cell Priming and T-Cell Dependent B-Cell Responses

    CD40/CD154 is an Essential Immune Pathway for T-Cell Priming and T-Cell Dependent B-Cell Responses • CD40 is expressed on the surface of • CD40 ligation on DCs induces cell maturation by promoting antigen dendritic cells, B-cells, antigen-presenting presentation and enhancing their costimulatory act

20. Slide 20: KPL-404 Single-Ascending-Dose Phase Study (KLH Challenge)

    KPL-404 Single-Ascending-Dose Phase 1 Study Part A (Single IV dose) Part B (Single SC Dose) KLH Challenge Groups* 10 mg/kg KLH Challenge d0 d4 d29 3 mg/kg 5 mg/kg 404 KLH KLH Weekly sampling for anti-KLH Ig 1 mg/kg 1 mg/kg 0.3 mg/kg 0.03 mg/kg** • Primary endpoints: Safety and Tolerability • Seconda

21. Slide 21: Final Data from KPL-404 Single-Ascending-Dose Phase Study: Pharmacokinetics

    Final Data from KPL-404 Single-Ascending-Dose Phase 1 Study Pharmacokinetic profiles for KPL-404 0.03 mg/kg IV 0.3 mg/kg IV 1 mg/kg IV KPL-404 Serum Concentration (µg/mL) 3 mg/kg IV 10 mg/kg IV 1 mg/kg SC Geometric mean ± SD 5 mg/kg SC Complete RO LLOQ First Detected IgG ADA: Visit Day 21 SD, standa

22. Slide 22: Final Data from KPL-404 Single-Ascending-Dose Phase Study: TDAR

    Final Data from KPL-404 Single-Ascending-Dose Phase 1 Study T-Cell Dependent Antibody Response (TDAR) for KLH antigen challenge Pooled placebo IV 1 mg/kg IV 3 mg/kg IV 10 mg/kg IV Pooled placebo SC 1 mg/kg SC KLH-Specific IgG Level (units/mL) 5 mg/kg SC Secondary KLH Challenge* Mean ± SE Primary KLH

23. Slide 23: KPL-404 Phase 2 Trial in Rheumatoid Arthritis

    KPL-404 Phase 2 Trial in Rheumatoid Arthritis Multiple-ascending-dose study that evaluates PK and safety and then transitions into a parallel dose efficacy portion PHARMACOKINETICS (PK) LEAD-IN PROOF-OF-CONCEPT Amended Cohort 3 PATIENT POPULATION: KPL-404 • Active RA who have an 5 mg/kg SC qwk inade

24. Slide 24: KPL-404 is a Potentially Best-in-Class, Subcutaneously Delivered Monoclonal Antibody

    KPL-404 is a Potentially Best-in-Class, Subcutaneously Delivered Monoclonal Antibody Inhibitor of the CD40/CD154 Interaction • KPL-404 drug product is formulated Anticipated in a high concentration liquid Therapeutic Range formulation that enables subcutaneous-administration • KPL-404 non-clinical a

25. Slide 25: KPL-404 Data Generated To Date Suggest it is Well-Positioned Against Competitors

    KPL-404 Data Generated To Date Suggest it is Well-Positioned Against Competitors Routes of Administration Company Product Phase Target Format Being Studied Key Dosing Regimens Being Studied KPL-404 P2 CD40 mAb SC 5mg/kg (~400mg) SC qwk / q2wk1 IV 10mg/kg IV q4wk ISCALIMAB P2 CD40 mAb SC Up to 300mg

26. Slide 26: Potential for Evaluation of KPL-404 in a Broad Range of Autoimmune Diseases

    Potential for Evaluation of KPL-404 in a Broad Range of Autoimmune Diseases CD40/CD154 interaction has been implicated in a number of devastating diseases Indications with Immune Thrombocytopenic Purpura* Published Data Kidney Transplant Myasthenia Gravis Sjogren’s Syndrome* Systemic Lupus Erythemat

27. Slide 27: Financials Second Quarter 2023

    Financials Second Quarter 2023

28. Slide 28: Second Quarter 2023 Financial Results

    Second Quarter 2023 Financial Results Income Statement Three Months Ended June 30, Collaboration Expenses1 Three Months Ended June 30, 2023 2022 2023 2022 Product Revenue $54.5M $27.0M ARCALYST Net Sales (RP + CAPS + DIRA) $54.5M $27.0M License and Collaboration Revenue $17.0M $0.0M Profit Split-Eli

29. Slide 29: Appendix ARCALYST

    Appendix ARCALYST (rilonacept)

30. Slide 30: Role of IL-1α and IL-1β in the Autoinflammatory Cycle of Recurrent Pericarditis

    Role of 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; I

31. Slide 31: 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

32. Slide 32: RHAPSODY Long-Term Extension Data Demonstrated Continued Treatment Response

    RHAPSODY Long-Term Extension Data Demonstrated Rilonacept Treatment Beyond 18 months Resulted in Continued Treatment Response1 1) Imazio M, Klein AL, et al. Prolonged Rilonacept Treatment in Rhapsody Long-term Extension Provided Persistent Reduction of Pericarditis Recurrence Risk. Poster 2223 (Pres

33. Slide 33: Appendix KPL-404

    © Appendix KPL-404

34. Slide 34: KPL-404 Does Not Cause Platelet Activation or Aggregation in vitro

    KPL-404 Does Not Cause Platelet Activation or Aggregation in vitro • At least three first-generation IgG1 anti-CD154 mAbs* were associated with thromboembolic events in humans and NHPs1 • Mechanism: Activation of platelets through cross-linking mediated by IgG-Fc/FcyRIIa interaction • Platelet activ

35. Slide 35: KPL-404 Showed Encouraging Results in a Non-Human Primate Model of TDAR

    KPL-404 Showed Encouraging Results in a Non-Human Primate Model of TDAR Mean KPL-404 PK Mean KPL-404 Receptor Occupancy (RO) Mean KLH IgG (Intravenous Dosing) 150 a n t i- K L H ( Ig G ) 0 m g/kg a n t i- K L H Ig G ( u g /m l) 1 m g/kg 5 m g/kg 10 m g/kg 100 50 0 P r e -d o se 7 14 21 28 T im e (d

36. Slide 36: Appendix Mavrilimumab

    © Appendix Mavrilimumab

37. Slide 37: Mavrilimumab, a GM-CSFRα antagonist, blocks GM-CSF signaling

    Mavrilimumab, a GM-CSFRα antagonist, blocks GM-CSF signaling; A Key Mediator of Inflammation and Autoimmunity • Granulocyte-macrophage colony stimulating factor (GM- mavrilimumab CSF) is a growth factor first identified as an inducer of differentiation and proliferation of myeloid cells (neutrophils

38. Slide 38: Corporate Presentation

    Corporate Presentation JULY 2023

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