Table of Contents UNITED STATES SECURITIES AND EXCHANGE COMMISSION Washington, D.C. 20549 FORM 10-Q (Mark One) ☒ QUARTERLY REPORT PURSUANT TO SECTION 13 OR 15(d) OF THE SECURITIES EXCHANGE ACT OF 1934 For the quarterly period ended March 31, 2021 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 ...

Table of Contents 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, 2020 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) (State or other jurisdi ...

Table of Contents UNITED STATES SECURITIES AND EXCHANGE COMMISSION Washington, D.C. 20549 FORM 10-Q (Mark One) ☒ QUARTERLY REPORT PURSUANT TO SECTION 13 OR 15(d) OF THE SECURITIES EXCHANGE ACT OF 1934 For the quarterly period ended September 30, 2020 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) (State or Othe ...

业绩总结 - Kiniksa预计在2020年向FDA提交针对复发性心包炎的补充生物制剂申请(sBLA)[6] - 复发性心包炎在美国的患病率约为4万名患者，其中约1.4万名患者对常规治疗反应不足[19] - 复发性心包炎的年复发率约为50%[17] - Kiniksa在复发性心包炎的市场机会中，预计初步可接触的患者为1.4万名[19] - Rilonacept在临床试验中，接受治疗的患者中，只有2名（6.7%）出现了复发，而安慰剂组中有23名（74.2%）患者出现复发[35] - Rilonacept组在16周内，81%的患者维持了临床反应，而安慰剂组仅为20%[36] - Rilonacept组的CRP正常化中位时间为7.0天（5.0, 8.0）[30] - Rilonacept治疗期间，心包炎发作的年化发生率从平均4.4次减少至0.18次[140] - 在Rilonacept治疗的患者中，CRP水平在第2周降至0.66 mg/dL，第24周降至0.09 mg/dL[142] 用户数据 - 参与试验的患者中，男性占43%，女性占57%[26] - 试验中，患者的平均年龄为44.7岁[26] - 在合格的患者中，67%接受了NSAIDs治疗，49%接受了类固醇治疗[27] - 参与者的生活质量评分显著改善，全球身体健康和心理健康的平均分数为60，远高于美国国家平均分50[127] - 在13名接受治疗的患者中，所有患者的心包症状均有所改善或消失[128] 新产品和新技术研发 - Kiniksa的Mavrilimumab针对巨细胞动脉炎的二期数据预计在2020年第四季度发布[6] - 针对COVID-19肺炎及超炎症反应的Mavrilimumab已在2020年第三季度启动适应性设计的二期/三期临床试验[6] - Kiniksa的KPL-404针对严重自身免疫疾病的第一阶段数据预计在2020年第四季度发布[6] - Mavrilimumab在GCA活检中显示GM-CSF和GM-CSFRα的水平较对照组显著升高，P<0.001[52] - Mavrilimumab治疗组在28天内100%患者达到临床改善终点，而对照组为65%（P=0.0001）[64] 市场扩张和并购 - GCA在美国的患病率约为75,000至150,000例[46] - 每年新发GCA患者约为8,000至20,000人[154] - Medicare覆盖的GCA患者约为61%[154] - 复发性心包炎的年发病率约为117,000例，初次复发患者约为26,000例[148] 负面信息 - Rilonacept在所有不良事件中，发生率为80.2%（69例），而安慰剂组为41.9%（13例）[38] - Rilonacept的药物相关不良事件（TEAEs）为53.5%（46例），安慰剂组为3.2%（1例）[38] - 在Rilonacept的随机撤回阶段，31名受试者中有10.0%（3人）报告外周水肿[118] - 在Rilonacept的随机撤回阶段，31名受试者中有3.3%（1人）报告心率增加[119] 其他新策略和有价值的信息 - 98%的患者表示需要额外的治疗以减少复发的可能性[16] - 预计治疗持续时间为6-9个月，长期可达12个月[39] - Rilonacept的未来监管里程碑总额为2750万美元[44] - Kiniksa将承担与额外适应症批准相关的100%开发费用[44]

业绩总结 - Kiniksa预计在2020年提交rilonacept用于复发性心包炎的补充生物制剂申请(sBLA)[6] - Mavrilimumab在2020年第四季度预计发布巨细胞动脉炎的第二阶段数据[6] 用户数据 - 复发性心包炎在美国的患病率约为4万，初步可接触的治疗机会约为1.4万[7] - 复发性心包炎患者的年复发率为50%[17] - 98%的复发性心包炎患者表示需要额外的治疗以减少复发的可能性[16] - 参与试验的患者中，86名患者的平均年龄为44.7岁，男性占43%[26] - 在合格的发作中，患者的CRP平均水平为6.18 mg/dL，NRS疼痛评分平均为6.2[28] 新产品和新技术研发 - Kiniksa的rilonacept获得了复发性心包炎的突破性疗法和孤儿药物认证[7] - Rilonacept在临床试验中显示出96%的相对风险降低，p值小于0.0001[34] - Mavrilimumab在动脉炎模型中相比对照组显著降低了动脉炎症，P<0.001[51] - Mavrilimumab在临床试验中显示出对CRP和IL-6的显著抑制，P<0.05[52] 市场扩张和并购 - Kiniksa的rilonacept在商业化后将与Regeneron进行50/50的利润分成[7] - Mavrilimumab的经济模型包括临床、监管和销售里程碑，以及基于年净销售额的分级特许权使用费[45] 未来展望 - 预计Rilonacept的治疗持续时间为6-9个月，长期可达12个月[39] - Mavrilimumab在COVID-19重症肺炎患者中显示出改善临床结果，包括更早的氧气撤离和缩短住院时间[60] - 预计美国可接触的Rilonacept患者总数约为14,000人[147] 负面信息 - Rilonacept在所有不良事件中，发生率为80.2%（69例），在随机撤回期为80.0%（24例），而安慰剂组为41.9%（13例）[38] - Rilonacept的严重不良事件（SAE）中，53.5%（46例）与药物相关，随机撤回期为33.3%（10例）[38] - 在Rilonacept治疗中，85%的患者成功停用或减少了皮质类固醇的使用[126] 其他新策略和有价值的信息 - Rilonacept组在前16周内，98%的天数无或轻微疼痛，而安慰剂组为46%[36] - 在治疗期间，CRP（C反应蛋白）水平显著降低，治疗期结束时的平均CRP为0.05 mg/dL[131] - 参与者的生活质量评分显著改善，全球身体健康和心理健康的平均分数为60，超过美国国家平均分50[126]

Table of Contents UNITED STATES SECURITIES AND EXCHANGE COMMISSION Washington, D.C. 20549 FORM 10-Q (Mark One) ☒ QUARTERLY REPORT PURSUANT TO SECTION 13 OR 15(d) OF THE SECURITIES EXCHANGE ACT OF 1934 For the quarterly period ended June 30, 2020 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 ...

FORM 10-Q (Mark One) Table of Contents UNITED STATES SECURITIES AND EXCHANGE COMMISSION Washington, D.C. 20549 ☒ QUARTERLY REPORT PURSUANT TO SECTION 13 OR 15(d) OF THE SECURITIES EXCHANGE ACT OF 1934 For the quarterly period ended March 31, 2020 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 ...

Table of Contents 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) (State or other jurisdi ...

Table of Contents UNITED STATES SECURITIES AND EXCHANGE COMMISSION Washington, D.C. 20549 FORM 10-Q (Mark One) ☒ QUARTERLY REPORT PURSUANT TO SECTION 13 OR 15(d) OF THE SECURITIES EXCHANGE ACT OF 1934 For the quarterly period ended September 30, 2019 OR Bermuda 98-1327726 (State or other jurisdiction of (I.R.S. Employer Kiniksa Pharmaceuticals, Ltd. Clarendon House 2 Church Street Hamilton HM11, Bermuda (808) 451-3453 (Address, zip code and telephone number, including area code of principal executive office ...

Table of Contents UNITED STATES SECURITIES AND EXCHANGE COMMISSION Washington, D.C. 20549 FORM 10-Q (Mark One) ☒ QUARTERLY REPORT PURSUANT TO SECTION 13 OR 15(d) OF THE SECURITIES EXCHANGE ACT OF 1934 For the quarterly period ended June 30, 2019 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 (808) 451-3453 (Address, zip code and telephone number, inc ...