 Rilonacept pivotal Phase 3 trial in recurrent pericarditis met statistical significance for primary and all major secondary efficacy endpoints; sBLA submission expected this year Mavrilimumab global Phase 23 adaptive design trial in severe COVID-19 pneumonia and hyperinflammation enrolling and dosing patients Data from mavrilimumab global Phase 2 trial in GCA and KPL-404 Phase 1 trial expected in Q4 2020 Initiation of dose-ranging Phase 2b trial of vixarelimab in prurigo nodularis expected in Q4 2020 Recent public offerings and concurrent private placements raised a total of 220 million in net proceeds; cash reserves expected to fund current operating plan into 2023 HAMILTON, Bermuda, July 30, 2020 GLOBE NEWSWIRE -- Kiniksa Pharmaceuticals, Ltd. Nasdaq: KNSA Kiniksa, a biopharmaceutical company focused on discovering, acquiring, developing and commercializing therapeutic medicines for patients with significant unmet medical need, today reported second quarter 2020 financial results and highlighted recent pipeline and corporate activity.Kiniksa had a brilliant first half of 2020, marked by statistically significant results from the pivotal Phase 3 study of rilonacept in recurrent pericarditis and the Phase 2a study of vixarelimab in prurigo nodularis as well as the COVID-19 mavrilimumab data published in The Lancet Rheumatology, said Sanj K. Patel, Chief Executive Officer and Chairman of the Board of Kiniksa. The second half of the year is also expected to be catalyst-filled. We are committed to submitting an sBLA to the FDA for rilonacept in recurrent pericarditis this year and look forward to bringing this potential treatment to patients as soon as possible. Our evaluations of mavrilimumab in severe COVID-19 pneumonia and vixarelimab in prurigo nodularis are also progressing, and we continue to expect data in the fourth quarter from the mavrilimumab GCA trial and from our anti-CD40 program, KPL-404. We expect the recent capital raises and continued financial discipline to extend our cash runway into 2023.Pipeline Activity Rilonacept IL-1α and IL-1β cytokine trap Kiniksa announced data from RHAPSODY, a global, randomized withdrawal design, pivotal Phase 3 clinical trial of rilonacept in recurrent pericarditis, a painful autoinflammatory cardiovascular disease. The primary efficacy endpoint of time-to-first adjudicated pericarditis recurrence in the randomized withdrawal period was highly statistically significant: rilonacept treatment resulted in a 96 reduction in risk of recurrent pericarditis events Hazard Ratio 0.04, p0.0001. All major secondary endpoints were also highly statistically significant. Based on the data from RHAPSODY, the Biologic License Application BLA for Cryopyrin-Associated Periodic Syndromes CAPS will transfer to Kiniksa from Regeneron Pharmaceuticals, Inc. Regeneron. Kiniksa plans to submit a supplemental Biologics License Application sBLA to the U.S. Food and Drug Administration FDA in recurrent pericarditis this year. Upon receipt of FDA approval for rilonacept in recurrent pericarditis, Kiniksa will assume the sales and distribution of rilonacept for the approved indications in the United States and evenly split profits on sales with Regeneron. Kiniksa announced the FDA granted Orphan Drug designation for rilonacept for the treatment of pericarditis, which includes recurrent pericarditis. Kiniksa is preparing for the commercialization of rilonacept in recurrent pericarditis by generating evidence on disease burden, building disease awareness with payers, physicians and advocacy groups, and establishing core capabilities such as distribution, patient services and data management.Mavrilimumab monoclonal antibody inhibitor targeting GM-CSFRα Kiniksa expects data from a global Phase 2 clinical trial of mavrilimumab in patients with giant cell arteritis GCA, a chronic inflammatory disease of medium-to-large arteries, in the fourth quarter of 2020. Kiniksa is enrolling and dosing the global, randomized, double-blind, placebo-controlled Phase 2 portion of an adaptive design Phase 23 clinical trial of mavrilimumab in severe coronavirus disease 2019 COVID-19 pneumonia and hyperinflammation. Additionally, a randomized, double-blind, placebo-controlled investigator-initiated study in the U.S. is enrolling and dosing patients. Kiniksa announced clinical outcomes data from the open-label treatment protocol1 with mavrilimumab in severe COVID-19 pneumonia and hyperinflammation. Mavrilimumab-treated patients experienced earlier and improved clinical outcomes compared to control-group patients, including earlier weaning from supplemental oxygen, shorter hospitalizations, and no deaths. These data were presented at the European E-Congress of Rheumatology EULAR 2020 and published in The Lancet Rheumatology. Kiniksa and Kite, a Gilead company, expect to commence a Phase 2 trial evaluating the investigational combination of Yescarta axicabtagene ciloleucel and mavrilimumab in relapsed or refractory large B-cell lymphoma in the second half of 2020. The objective of the trial is to determine the effect of mavrilimumab on the safety of Yescarta. Preclinical evidence shows the potential for interruption of granulocyte macrophage colony stimulating factor GM-CSF signaling to disrupt chimeric antigen receptor T CAR T cell-mediated inflammation without disrupting anti-tumor efficacy.Vixarelimab monoclonal antibody inhibitor of signaling through OSMRβ Kiniksa expects to initiate a dose-ranging Phase 2b clinical trial of vixarelimab in prurigo nodularis, a chronic inflammatory skin condition, in the fourth quarter of 2020. Kiniksas Phase 2a clinical trial of vixarelimab in patients with prurigo nodularis met its primary efficacy endpoint: the reduction in weekly-average Worst-Itch Numeric Rating Scale WI-NRS from baseline at Week 8 was statistically significantly greater in patients who received vixarelimab versus those who received placebo. Additionally, a statistically significantly greater percentage of vixarelimab recipients achieved a prurigo nodularis-investigators global assessment PN-IGA score of 01 at Week 8 compared to placebo recipients. Kiniksa announced data from an exploratory Phase 2 trial of vixarelimab in diseases characterized by chronic pruritus, including plaque psoriasis, chronic idiopathic pruritus, lichen simplex chronicus, chronic idiopathic urticaria and lichen planus. The plaque psoriasis cohort achieved a statistically significant reduction in weekly-average WI-NRS at Week 8. Additionally, the lichen simplex chronicus, chronic idiopathic urticaria and lichen planus cohorts showed encouraging efficacy results as measured by percent change from baseline in weekly-average WI-NRS at Week 8.KPL-404 monoclonal antibody inhibitor of signaling between CD40 and CD40L Kiniksa expects data from a single-ascending-dose Phase 1 clinical trial of KPL-404 in healthy volunteers in the fourth quarter of 2020. The first-in-human trial will provide safety data and pharmacokinetics as well as receptor occupancy and T-cell Dependent Antibody Response TDAR.Corporate Activity In May of 2020, Kiniksa completed a public offering of 2,760,000 Class A common shares at a public offering price of 18.25 per share, which included the exercise in full by the underwriters of their option to purchase additional shares. Concurrent with the public offering, Kiniksa sold 1,600,000 Class A1 common shares to certain existing shareholders affiliated with certain of Kiniksas directors at a sale price equal to the price of the public offering. The aggregate net proceeds to Kiniksa from these offerings after deducting underwriting discounts, commissions and other offering costs were approximately 74.5 million. In July of 2020, Kiniksa completed a public offering of 5,952,381 Class A common shares at a public offering price of 21.00 per share. Concurrent with the public offering, Kiniksa sold 1,428,572 Class A1 common shares to certain existing shareholders affiliated with certain of Kiniksas directors at a sale price equal to the price of the public offering. The estimated aggregate net proceeds to Kiniksa from these offerings after deducting underwriting discounts, commissions and other offering costs were approximately 145.9 million.Financial Results For the second quarter of 2020, Kiniksa reported a net loss of 37.5 million, compared to a net loss of 37.2 million for the second quarter of 2019. Total operating expenses for the second quarter of 2020 totaled 31.9 million, compared to 39.3 million for the second quarter of 2019. Non-cash share-based compensation expense totaled 4.9 million for the second quarter of 2020, compared to 3.5 million for the second quarter of 2019. As of June 30, 2020, the company had cash, cash equivalents and short-term investments of 252.4 million and no debt. Kiniksa had proforma cash reserves2 of 398.3 million as of June 30, 2020, which includes approximately 252.4 million of cash, cash equivalents and short-term investments as of June 30, 2020 and approximately 145.9 million of estimated net proceeds from our July 2020 public offering and concurrent private placement as though we had closed on these financings in the second quarter of 2020.Financial Guidance Kiniksa expects that its cash, cash equivalents and short-term investments, inclusive of the estimated net proceeds from its recent public offering and concurrent private placement, will fund its current operating plan into 2023. 