Kiniksa Pharmaceuticals Reports Second Quarter 2022 Financial Results and Provides Corporate Update - ARCALYST (rilonacept) net revenue of $27.0 million in Q2 2022 - - ARCALYST full-year 2022 net revenue expected to be $

Kiniksa Pharmaceuticals

Reports Second Quarter 2022 Financial Results and Provides Corporate Update

ARCALYST (rilonacept) net revenue of $27.0 million in Q2 2022 -

ARCALYST full-year 2022 net revenue expected to be $115 - $130 million -

- Upfront and near-term proceeds of $100 million expected from vixarelimab global license agreement -

reserves after the close of the vixarelimab global license agreement expected to fund operations into at least 2025 -

Conference call and webcast scheduled for 8:30 am ET today -

- August 3, 2022 - 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 second quarter 2022 financial

results and provided a corporate update.

"The continued momentum of ARCALYST in recurrent pericarditis

in the second quarter of 2022 provides conviction in our full-year expectation for net revenue of between $115 to 130 million. Additionally,

we believe the strong performance of ARCALYST since launch supports incremental investment to broaden our reach and help even more patients

suffering from recurrent pericarditis," said Sanj K. Patel, Chairman and Chief Executive Officer of Kiniksa. "We are also

focused on expanding our portfolio by leveraging our cross-functional cardiovascular expertise. These efforts will be enabled in part

by the non-dilutive proceeds from our global license agreement with Genentech."

ARCALYST (IL-1 and IL-1 cytokine trap)

KPL-404 (monoclonal antibody inhibitor of CD40-CD154 interaction)

Mavrilimumab (monoclonal antibody inhibitor targeting GM-CSFR )

Conference Call Information

Kiniksa 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 portfolio assets, ARCALYST, KPL-404, and mavrilimumab, are based on strong

biologic rationale or validated mechanisms, target underserved conditions, and offer the potential for differentiation. These assets are

designed to modulate immunological pathways across a spectrum of diseases. For more information, please visit www.kiniksa.com.

ARCALYST is a weekly, subcutaneously

injected recombinant dimeric fusion protein that blocks interleukin-1 alpha (IL-1 ) and interleukin-1 beta (IL-1 ) signaling.

ARCALYST was discovered by Regeneron and is approved by the U.S. Food and Drug Administration (FDA) for recurrent pericarditis, cryopyrin-associated

periodic syndromes (CAPS), including Familial Cold Autoinflammatory Syndrome and Muckle-Wells Syndrome, and deficiency of IL-1 receptor

antagonist (DIRA). The FDA granted Breakthrough Therapy designation to ARCALYST for the treatment of recurrent pericarditis in 2019 and

Orphan Drug designation to ARCALYST for the treatment of pericarditis in 2020. The European Commission granted Orphan Drug Designation

to ARCALYST for the treatment of idiopathic pericarditis in 2020.

ARCALYST is indicated for:

IMPORTANT SAFETY INFORMATION

For more information about

ARCALYST, talk to your doctor and see the Product Information.

KPL-404 is an investigational

humanized monoclonal antibody that is designed to inhibit CD40-CD154 (CD40 ligand) 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-CD154 interaction

is an attractive approach to address multiple autoimmune disease pathologies.

investigational fully human monoclonal antibody that blocks activity of GM-CSF by specifically binding to the alpha subunit of the

GM-CSF receptor (GM-CSFR ). Phase 2 clinical trials of mavrilimumab in rheumatoid arthritis and GCA achieved their primary and

secondary endpoints with statistical significance. Kiniksa is evaluating the development of mavrilimumab in rare cardiovascular

diseases where the GM-CSF mechanism has been implicated and that have synergies with the company's existing commercial

Forward-Looking Statements

This 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: the global license agreement

between Kiniksa and Genentech, including (i) anticipated upfront, near-term, milestone and royalty payments under such agreement, (ii)

statements regarding the use of proceeds from the agreement and (iii) Kiniksa's plan to complete its Phase 2b clinical trial of

vixarelimab in prurigo nodularis; our expectation that ARCALYST net revenue for full-year 2022 will be between $115 million and $130 million;

our plans to evolve our sales operation with approximately 20 additional field sales representatives in the fourth quarter of 2022; our

expectation about our cash reserves funding our current operating plan into 2025 following the close of the vixarelimab global license

agreement with Genentech; our expectations regarding our next steps for mavrilimumab; our beliefs about the mechanisms of action of our

product candidates and potential impact of their approach, including that using KPL-404 to disrupt the CD40-CD154 interaction is an attractive

approach to address multiple autoimmune disease pathologies; and our belief that all of our product candidates offer the potential for

These 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: ours and Genentech's ability to obtain antitrust clearance and close our global license agreement in a timely

manner; our ability to realize anticipated near-term payments, milestones and royalty payments under such agreement; our ability to

successfully utilize the proceeds we will receive from such agreement; delays or difficulty in enrollment of patients in, and

activation or continuation of sites for, our clinical trials; delays or difficulty in completing our clinical trials as originally

designed; potential for changes between final data and any preliminary, interim, top-line or other data from clinical trials; our

inability to replicate results 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; potential undesirable

side effects caused by our products and product candidates; our inability to demonstrate safety and efficacy to the satisfaction of

applicable regulatory authorities; potential for applicable regulatory authorities to not accept our filings, delay or deny approval

of any of our product candidates or require additional data or trials to support approval; inability to successfully execute on our

commercial strategy for ARCALYST; our reliance on third parties as the sole source of supply of the drug substance and drug product

used in our products and product candidates; our reliance on Regeneron as the sole manufacturer of ARCALYST; raw materials,

important ancillary products and drug substance and/or drug product shortages; our reliance on third parties to conduct research,

clinical trials, and/or certain regulatory activities for our product candidates; complications in coordinating requirements,

regulations and guidelines of regulatory authorities across jurisdictions for our clinical trials; the impact of the COVID-19

pandemic and any subsequent pandemic and measures taken in response to such pandemics on our business and operations as well as the

business and operations of our manufacturers, CROs upon whom we rely to conduct our clinical trials, and other third parties with

whom we conduct business or otherwise engage, including the FDA and other regulatory authorities; changes in our operating plan and

funding requirements; and existing or new competition.

These and other important factors discussed in our filings with the

U.S. Securities and Exchange Commission, including under the caption "Risk Factors" contained therein, could cause actual

results to differ materially from those indicated by the forward-looking statements made in this press release. Any such forward-looking

statements represent management's estimates as of the date of this press release. Except as required by law, we disclaim any intention

or obligation to update or revise any forward-looking statements. These forward-looking statements should not be relied upon as representing

our views as of any date subsequent to the date of this press release.

ARCALYST is a registered trademark of Regeneron

Pharmaceuticals, Inc. All other trademarks are the property of their respective owners.

Every Second Counts!

Kiniksa Investor and Media Contact