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KINIKSA Kiniksa Reports First Quarter 2021 Financial Results and Recent Corporate and Portfolio Activity May 4 , 2021 -ARCALYSTⓇ ( rilonacept ) launched as the first and only FDA - approved therapy for recurrent pericarditis - - Mavrilimumab Phase 2 severe COVID - 19 data demonstrated a reduction in mechanical ventilation and death at Day 29 ; Phase 3 enrollment ongoing - Final KPL - 404 Phase 1 data support further development in patients ; Phase 2 proof - of - concept trial initiation planned for 2H 2021- - Cash reserves of approximately $ 264 million - HAMILTON , Bermuda , May 04 , 2021 ( GLOBE NEWSWIRE ) -- Kiniksa Pharmaceuticals . Ltd. ( Nasdaq : KNSA ) ( " Kiniksa " ) , a biopharmaceutical company with a portfolio of assets designed to modulate immunological pathways across a spectrum of diseases , today reported first quarter 2021 financial results and recent corporate and portfolio activity . " The first quarter was transformational for Kiniksa with the approval of ARCALYST as the first and only FDA - approved therapy for patients with recurrent pericarditis . We are focused on the launch of ARCALYST and are confident in our commercialization strategy , " said Sanj K. Patel , Chief Executive Officer and Chairman of the Board of Kiniksa . " Additionally , we are executing across our broader portfolio of immune - modulating assets . We recently reported positive data for mavrilimumab in severe COVID - 19 pneumonia and hyperinflammation and remain engaged with the FDA and other government agencies to identify pathways for accelerated availability of mavrilimumab as a potential therapeutic option for this patient population . We also reported positive final Phase 1 data for our potentially best - in - class anti - CD40 program , KPL - 404 , and plan to initiate a Phase 2 proof - of - concept trial in the second half of 2021. " Portfolio Activity ARCALYST ( IL - 1a and IL - 1ẞ cytokine trap ) • Kiniksa received approval from the U.S. Food and Drug Administration ( FDA ) on March 18 , 2021 , for ARCALYST for the treatment of recurrent pericarditis and reduction in risk of recurrence in adults and children 12 years and older . The commercial launch of ARCALYST in recurrent pericarditis commenced in April 2021 . • Kiniksa is responsible for sales and distribution of ARCALYST for all the approved indications in the United States , including cryopyrin - associated periodic syndromes ( CAPS ) and deficiency of IL - 1 receptor antagonist ( DIRA ) , and will evenly split profits with Regeneron Pharmaceuticals , Inc. ( Regeneron ) . • Kiniksa is executing on its commercial strategy , including engagement with priority accounts and payers to enable rapid and broad access to ARCALYST for patients . • Kiniksa is enrolling pediatric and adult patients with recurrent pericarditis in the RESONANCE registry . Mavrilimumab ( monoclonal antibody inhibitor targeting GM - CSFRα ) • Kiniksa expects to provide next steps for mavrilimumab , including for giant cell arteritis ( GCA ) , in the second quarter of 2021 . • Kiniksa announced data from the Phase 2 portion of the Phase 2/3 clinical trial of mavrilimumab in non - mechanically ventilated patients with severe COVID - 19 pneumonia and hyperinflammation receiving local standard of care . Non - mechanically ventilated patients treated with mavrilimumab demonstrated a reduction in mechanical ventilation and death at Day 29 pooled across dose cohorts . • Kiniksa continues to advance its engagement activities with the FDA and other government agencies to identify pathways to potentially accelerate availability of mavrilimumab as a therapeutic option for severe COVID - 19 patients . Enrollment in the Phase 3 portion of the trial is ongoing . Vixarelimab ( monoclonal antibody inhibitor of signaling through OSMRẞ ) • Kiniksa is conducting a placebo - controlled Phase 2b clinical trial of vixarelimab in prurigo nodularis , evaluating a range of once - monthly dose regimens via subcutaneous ( SC ) injection . • The primary efficacy endpoint is the percent change from baseline in the weekly - average Worst - Itch Numeric Rating Scale at Week 16 . KPL - 404 ( monoclonal antibody inhibitor of signaling between CD40 and CD154 ) • Kiniksa announced final data today from the KPL - 404 Phase 1 clinical trial in healthy volunteers . KPL - 404 was well