Kiniksa Highlights Corporate Priorities and Expected 2021 Milestones - PDUFA goal date of

Highlights Corporate Priorities and Expected 2021 Milestones

PDUFA goal date of March 21, 2021 for rilonacept in recurrent pericarditis -

Data from Phase 2 portion of mavrilimumab Phase 2/3 trial in severe COVID-19 pneumonia and hyperinflammation expected in 1H 2021

Final Phase 1 KPL-404 data expected in 1H 2021 -

BERMUDA - January 11, 2021 -

Kiniksa Pharmaceuticals, Ltd. (Nasdaq: KNSA) (Kiniksa), a biopharmaceutical company with a pipeline of assets designed

to modulate immunological pathways across a spectrum of diseases, today highlighted its corporate priorities and expected 2021

milestones. Sanj K. Patel, Chief Executive Officer and Chairman of the Board of Kiniksa will provide further detail in a corporate

presentation at the Virtual 39th Annual J.P. Morgan Healthcare Conference today, Monday, January 11, 2021 at 4:30 p.m.

was marked by significant progress across our entire pipeline, setting the stage to build long-term value across our portfolio,"

said Sanj K. Patel, Chief Executive Officer and Chairman of the Board of Kiniksa. "Moving forward, 2021 has the potential

to be a transformational year for Kiniksa with multiple catalysts expected across our pipeline, notably with the potential commercial

launch of rilonacept in recurrent pericarditis in the first half of the year. As we focus on our launch readiness preparations,

our commitment to bringing novel therapies to patients with unmet need remains at the core of our goals."

(IL-1 and IL-1 cytokine trap)

(monoclonal antibody inhibitor targeting GM-CSFR )

(monoclonal antibody inhibitor of signaling through OSMR )

(monoclonal antibody inhibitor of signaling between CD40 and CD40L)

ended 2020 with approximately $323 million in cash, cash equivalents and short-term investments (unaudited). The company expects

that these cash reserves will fund its current operating plan into 2023.

at the Virtual 39th Annual J.P. Morgan Healthcare Conference

will webcast its corporate presentation at the Virtual 39th Annual J.P. Morgan Healthcare Conference today, Monday,

January 11, 2021 at 4:30 p.m. Eastern Time. A live webcast of Kiniksa's presentation will be accessible through the Investors

& Media section of the company's website (www.kiniksa.com). A replay of the webcast will be available on Kiniksa's

website for 14 days following the conference.

is a biopharmaceutical company focused on discovering, acquiring, developing and commercializing therapeutic medicines for patients

suffering from debilitating diseases with significant unmet medical need. Kiniksa's product candidates, rilonacept, mavrilimumab,

vixarelimab and KPL-404, are based on strong biologic rationale or validated mechanisms, target underserved conditions and offer

the potential for differentiation. These pipeline assets are designed to modulate immunological pathways across a spectrum of

diseases. For more information, please visit www.kiniksa.com.

is a weekly, subcutaneously-injected, recombinant dimeric fusion protein that blocks interleukin-1 alpha (IL-1 ) and interleukin-1

beta (IL-1 ) signaling. Rilonacept was discovered and developed by Regeneron and is approved by the FDA under the brand name

ARCALYST for the treatment of CAPS, specifically Familial Cold Autoinflammatory Syndrome and Muckle-Wells

Syndrome, and DIRA. Rilonacept in recurrent pericarditis is an investigational drug. The FDA granted Breakthrough Therapy designation

to rilonacept for the treatment of recurrent pericarditis in 2019 and Orphan Drug designation to rilonacept for the treatment

of pericarditis in 2020.

information about ARCALYST (rilonacept) Injection

blockade may interfere with immune response to infections. Serious, life-threatening infections have been reported in patients

taking ARCALYST. ARCALYST should be discontinued if a patient develops a serious infection. Taking ARCALYST with TNF inhibitors

is not recommended because this may increase the risk of serious infections.

Patients should not receive a live vaccine while taking ARCALYST. It is recommended that prior to initiation of therapy with ARCALYST

patients receive all recommended vaccinations, as appropriate, including pneumococcal vaccine and inactivated influenza vaccine.

In the initial development program for ARCALYST, six serious adverse reactions were reported by four patients: Mycobacterium intracellular

infection, gastrointestinal bleeding and colitis, sinusitis and bronchitis and Streptococcus pneumoniae meningitis. The most commonly

reported adverse reactions associated with ARCALYST were injection site reaction and upper respiratory tract infection. Patients

should be monitored for changes in their lipid profiles and provided with medical treatment if warranted. Treatment with immunosuppressants,

including ARCALYST, may result in an increase in risk of malignancies. Hypersensitivity reactions associated with ARCALYST administration

in clinical studies have been rare. If a hypersensitivity reaction occurs, administration of ARCALYST should be discontinued and

appropriate therapy initiated.

is an investigational fully-human monoclonal antibody that targets granulocyte macrophage colony stimulating factor receptor alpha

(GM-CSFR ). Mavrilimumab was dosed in over 550 patients with rheumatoid arthritis through Phase 2b clinical studies in Europe and

achieved prospectively-defined primary endpoints of efficacy and safety. Kiniksa's lead indication for mavrilimumab is GCA,

a rare inflammatory disease of medium-to-large arteries. Kiniksa is also evaluating mavrilimumab in COVID-19 pneumonia and hyperinflammation.

The FDA granted Orphan Drug designation to mavrilimumab for the treatment of GCA in 2020.

is an investigational fully-human monoclonal antibody that targets oncostatin M receptor beta (OSMR ),

which mediates signaling of interleukin-31 (IL-31) and oncostatin M (OSM), two key cytokines implicated in pruritus,

inflammation and fibrosis. Kiniksa believes vixarelimab to be the only monoclonal antibody in development that targets both

pathways simultaneously. Kiniksa's lead indication for vixarelimab is prurigo nodularis, a chronic inflammatory skin

condition characterized by severely pruritic skin nodules. The FDA granted Breakthrough Therapy designation to

vixarelimab for the treatment of pruritus associated with prurigo nodularis in 2020.

KPL-404 is an investigational humanized monoclonal antibody that is designed to inhibit

CD40-CD40 ligand (CD40L) interaction, a key T-cell co-stimulatory signal critical

for B-cell maturation and immunoglobulin class switching and Type 1 immune responses. Kiniksa believes disrupting the CD40-CD40L

interaction is an attractive approach for multiple autoimmune disease pathologies such as rheumatoid arthritis, Sjogren's

syndrome, Graves' disease, systemic lupus erythematosus and solid organ transplant. Kiniksa owns or controls the intellectual

property related to KPL-404.

press release contains forward-looking statements within the meaning of the Private Securities Litigation Reform Act of 1995.

In some cases, you can identify forward looking statements by terms such as "may," "will," "should,"

"expect," "plan," "anticipate," "could," "intend," "target,"

"project," "contemplate," "believe," "estimate," "predict," "potential"

or "continue" or the negative of these terms or other similar expressions, although not all forward-looking statements

contain these identifying words. All statements contained in this press release that do not relate to matters of historical

fact should be considered forward-looking statements, including without limitation, statements regarding: our belief that progress

made across our pipeline in 2020, has set the stage to build long-term value across our portfolio; our belief that 2021 has the

potential to be a transformational year for Kiniksa; expected multiple catalysts across our pipeline in 2021; the potential commercial

launch of rilonacept in recurrent pericarditis in the first half of the year, if approved by the FDA; expected timing of data

from clinical trials, including expected data from the Phase 2 portion of the adaptive design Phase 2/3 clinical trial of mavrilimumab

in severe COVID-19 pneumonia and hyperinflammation in the first half of 2021, next steps for the development of mavrilimumab,

including for giant cell arteritis (GCA), in the first half of 2021, and final data from the single-ascending-dose Phase 1 clinical

trial of KPL-404 in healthy volunteers in the first half of 2021; our belief that KPL-404 has the potential to address a broad

range of autoimmune diseases; our beliefs about the mechanisms of action of our product candidates and potential impact of their

approach, including our beliefs that vixarelimab is the only monoclonal antibody in development

that targets both interleukin-31 (IL-31) and oncostatin M (OSM) pathways simultaneously; that KPL-404's disruption

of the CD40-CD40L interaction is an attractive approach for multiple autoimmune disease pathologies; our belief that all of our

product candidates offer the potential for differentiation; and expectation about our cash reserves funding our current operating

forward-looking statements are based on management's current expectations. These statements are neither promises nor

guarantees, but involve known and unknown risks, uncertainties and other important factors that may cause our actual results,

performance or achievements to be materially different from any future results, performance or achievements expressed or

implied by the forward-looking statements, including without limitation, the following: delays or difficulty in enrollment of

patients in, and activation or continuation of sites for, our clinical trials; amendments to our clinical trial

protocols initiated by us or required by regulatory authorities; delays or difficulty in completing our clinical trials,

including as a result of the COVID-19 pandemic; potential for changes between final data and any preliminary, interim,

top-line or other data from clinical trials conducted by us or third parties; our inability to replicate in later clinical

trials the positive final data from our earlier clinical trials or studies; impact of additional data from us or other

companies, including the potential for our data to produce negative, inconclusive or commercially uncompetitive results;

impact of additional data from us or other companies; potential undesirable side effects caused by our product candidates;

our inability to demonstrate safety and efficacy to the satisfaction of applicable regulatory authorities or otherwise

producing negative, inconclusive or commercially uncompetitive results; potential for applicable regulatory authorities to

not accept our BLA or sBLA filings or to delay or deny approval of any of our product candidates or to require additional

trials to support any such approval; our reliance on third parties as the sole source of supply of the drug substance and