KINIKSA PHARMACEUTICALS KINIKSA ANNUAL REPORT 2019 RELENTLESS. PASSIONATE. FOCUSED.™ 2019 ANNUAL REPORT ANNUAL 2019 Every Second Counts! ™ Dear Fellow Shareholders, MANAGEMENT TEAM WEBSITE Sanj K. Patel* Kiniksa.com I hope this letter finds you well as we adjust our routines and • We are evaluating KPL-404 in a single-ascending-dose Chief Executive Officer & Chairman of the Board navigate the impact of the coronavirus. At Kiniksa, the health trial in healthy volunteers to measure safety data and LEGAL COUNSEL and safety of our employees as well as the patients and people pharmacokinetics as well as receptor occupancy and John F. Paolini, MD, PhD* Senior Vice President, Chief Medical Officer Latham & Watkins LLP participating in and operating our clinical trials are of paramount T-cell Dependent Antibody Response (TDAR). The CD40- Boston, Massachusetts importance. We entered 2020 with our clinical-stage assets having CD40L pathway has been established in a broad range Thomas Beetham* the potential to generate data-driven value; these milestones of autoimmune diseases, such as rheumatoid arthritis, Executive Vice President, Corporate Development & Operations, Conyers Dill & Pearman Limited Chief Legal Officer Hamilton, HM CX, Bermuda remain on track thanks to the utmost dedication of our team. We will Sjogren’s syndrome, Graves’ disease, systemic lupus continue to monitor the pandemic and plan to adapt as necessary. erythematosus and solid organ transplant. Qasim Rizvi* Senior Vice President, Operations & Chief Commercial Officer INDEPENDENT REGISTERED ACCOUNTING FIRM Kiniksa is a clinical-stage biopharmaceutical company focused In 2019, each of our clinical-stage assets posted key accomplishments. PricewaterhouseCoopers LLP on developing immune-modulating medicines for the benefit Eben Tessari Boston, Massachusetts Senior Vice President, Chief Business Officer of patients suffering from devastating diseases. Through highly- • Rilonacept: The FDA granted Breakthrough Therapy dedicated clinical and corporate execution, 2019 represented a designation for rilonacept for the treatment of recurrent Dave Nichols TRANSFER AGENT AND REGISTRAR significant step toward this vision. pericarditis. Additionally, we reported final data from an open- Senior Vice President, Technical Operations American Stock Transfer & Trust Company, LLC label Phase 2 trial of rilonacept in recurrent pericarditis. The Brooklyn, New York Dana Martin Our clinical-stage assets are based on strong biologic rationale data provided first evidence that rilonacept treatment in the Senior Vice President, Global Medical Affairs and/or validated mechanisms. study improved clinically meaningful outcomes associated STOCK INFORMATION Martina Struck, PhD with the unmet medical need in recurrent pericarditis. We also Nasdaq Global Select Market: KNSA • Rilonacept is an inhibitor of interleukin-1 alpha (IL-1 т) and achieved target enrollment on time for our pivotal Phase 3 trial Vice President, Regulatory Affairs interleukin-1 beta (IL-1у), cytokines that have been shown in recurrent pericarditis (RHAPSODY) and continued to prepare Mike Megna INVESTOR RELATIONS to play a role in inflammatory diseases. for commercialization by generating evidence on disease Vice President, Finance & Chief Accounting Officer Mark Ragosa burden, building disease awareness with payers, • Mavrilimumab is a monoclonal antibody inhibitor of Melissa Manno Vice President, Investor Relations granulocyte macrophage colony stimulating factor (GM- physicians and advocacy groups and establishing core Vice President, Human Resources [email protected] CSF) receptor signaling. GM-CSF is a key growth factor capabilities such as distribution and patient services. and cytokine in autoinflammation and autoimmunity. * Executive officers as defined under Rule 3b-7 under the This Annual Report contains forward-looking statements that involve risks, • Mavrilimumab: We achieved target enrollment on time for Securities Exchange Act of 1934, as amended. uncertainties and other important factors that could cause results to differ • Vixarelimab (KPL-716) is a monoclonal antibody inhibitor our Phase 2 trial in GCA and initiated a clinical collaboration materially from those projected. In some cases, you can identify these statements with Kite, a Gilead company, to evaluate the investigational by terms such as “may,” “will,” “should,” “expect,” “plan,” “anticipate,” “could,” of signaling through oncostatin M receptor beta (OSMRу), BOARD OF DIRECTORS “intend,” “goal,” “design,” “target,” “project,” “contemplate,” “believe,” “estimate,” combination of Yescarta® (axicabtagene ciloleucel) and “predict,” “potential” or “continue” or their negative or other similar expressions. the shared receptor subunit for interleukin-31 (IL-31) and Chairman mavrilimumab in relapsed or refractory large B-cell lymphoma. These important factors include those discussed in our Annual Report on Form oncostatin M (OSM) signaling. IL-31 and OSM are key Sanj K. Patel 10-K for the year ended December 31, 2019 (which forms a part of this Annual cytokines implicated in inflammation, pruritus, and fibrosis. Chief Executive Officer Report) under the caption “Risk Factors.” Accordingly, you are cautioned not to • Vixarelimab: Interim repeated-single-dose Phase 1b data place undue reliance on such statements. We undertake no obligation to update • KPL-404 is a monoclonal antibody inhibitor of the for vixarelimab in subjects with moderate-to-severe atopic Lead Independent Director any forward-looking statements. CD40-CD40 ligand (CD40L) interaction, a key T-cell dermatitis showed a rapid and sustained anti-pruritic Felix J. Baker, PhD co-stimulatory signal critical for B-cell maturation and response as measured by weekly average Worst-Itch Co-Managing Member, Baker Bros. Advisors LP Numeric Rating Scale (WI-NRS). These results supported immunoglobulin class switching. Directors further development of vixarelimab in a Phase 2a trial Additionally, our clinical-stage assets target diseases with significant in prurigo nodularis and an exploratory Phase 2 trial in Stephen R. Biggar, MD, PhD Partner, Baker Bros. Advisors LP unmet medical need and offer the potential for differentiation. diseases characterized by chronic pruritus. Richard S. Levy, MD • We are evaluating rilonacept for the potential treatment • KPL-404: The FDA accepted our investigational new drug Biopharmaceutical Consultant of recurrent pericarditis, a painful autoinflammatory application for the clinical study of KPL-404, and we initiated Thomas R. Malley cardiovascular disease, in a pivotal Phase 3 trial. There a Phase 1 trial. President, Mossrock Capital, LLC are no U.S. Food and Drug Administration (FDA)-approved therapies for recurrent pericarditis. In 2020, our clinical-stage assets are expected to generate clinical Tracey L. McCain data, including pivotal Phase 3 data from rilonacept, Phase 2 data Executive Vice President, Chief Legal and • We are evaluating mavrilimumab for the potential treatment from mavrilimumab, Phase 2 data from vixarelimab, and Phase 1 Compliance Officer, Blueprint Medicine Corporation of giant cell arteritis (GCA), a chronic inflammatory disease data from KPL-404. We believe these data have the potential to Kimberly J. Popovits of medium-to-large arteries, in a Phase 2 trial. While help inform our portfolio strategy and capital allocation decisions. Former Chief Executive Officer & Chairman of the Board, there is one FDA-approved therapy for GCA, we believe Genomic Health, Inc. mavrilimumab’s mechanism of action acts upstream and that As we continue to execute toward our goal of becoming a generational biopharmaceutical company, we will keep science Barry D. Quart, PharmD an unmet medical need remains. We are also exploring the President, Chief Executive Officer & Director, and patients at the center of our decisions. Thank you for your potential for mavrilimumab to treat a host of vasculitides and Heron Therapeutics, Inc. oncologic indications. ongoing support. ADDRESS • We are evaluating vixarelimab for the potential treatment of Every Second Counts!™ Kiniksa Pharmaceuticals, Ltd. prurigo nodularis, a chronic inflammatory skin condition, in Unless otherwise expressly stated, we obtained the industry, business, market and Clarendon House a Phase 2a trial. There are no FDA-approved therapies for other data contained in this Annual Report from reports, research surveys, clinical 2 Church Street trials, studies and similar data prepared by market research firms and other third prurigo nodularis. We are also investigating the potential Hamilton HM 11 parties, from industry, medical and general publications, and from government Bermuda data and similar sources. for vixarelimab to treat diseases characterized by chronic Sanj K. Patel pruritus in an exploratory Phase 2 trial. CEO and Chairman of the Board UNITED STATES SECURITIES AND EXCHANGE COMMISSION Washington, D.C. 20549 FORM 10-K (Mark One) ☒ ANNUAL REPORT PURSUANT TO SECTION 13 OR 15(d) OF THE SECURITIES EXCHANGE ACT OF 1934 For the fiscal year ended December 31, 2019 OR ☐ TRANSITION REPORT PURSUANT TO SECTION 13 OR 15(d) OF THE SECURITIES EXCHANGE ACT OF 1934 For the transition period from to Commission file number: 001-38492 Kiniksa Pharmaceuticals, Ltd. (Exact name of registrant as specified in its charter) Bermuda 98-1327726 (State or other jurisdiction of (I.R.S. Employer incorporation or organization) Identification Number) Kiniksa Pharmaceuticals, Ltd. Clarendon House 2 Church Street Hamilton HM11, Bermuda + (44) 808-189-6257