Kiniksa Pharmaceuticals, Ltd.

Thank you for standing by and welcome to the Conexa Pharmaceutical Second Quarter 2025 Earnings Conference Call. At this time, all participants are in listen-only mode. After the speaker's presentation, there will be a question and answer session. To ask a question during this session, you'll need to press star 1-1 on your telephone. If your question has been answered and you'd like to remove yourself from the queue, simply press star 1-1 again. As a reminder, today's program is being recorded. And now I'd like to introduce your host for today's program, Jonathan Kirschenbaum, Investor Relations. Please go ahead, sir.

Thank you, operator. Good morning, everyone, and thank you for joining Conexa's call to discuss our second quarter 2025 financial results and recent portfolio execution. A press release highlighting these results can be found on our website under the Investors section. As for the agenda for today's call, our Chief Executive Officer, Sanj K Patel, will start with an introduction and overview of our business. Ross Moat, Conexa's Chief Commercial Officer, will provide an update on ARCALYST's commercial execution. Then, Mark Legosa, our Chief Financial Officer, will review our second quarter 2025 financial results. Finally, Sanj will share closing remarks and kick off the Q&A session, for which John F. Paolini, our Chief Medical Officer, and Evan Tesari, our Chief Operating Officer, will also be on the line. Before getting started, please note that we will be making forward-looking statements today that are subject to risks and uncertainties that may cause actual results to differ materially from these statements. A review of such statements and risk factors can be found on this slide, as well as under the caption, risk factors, contained in our SEC filings. These statements speak only at the date of this presentation, and we undertake no obligation to update such statements, except as required by law. With that, I will turn it over to Sanj.

Thanks, Jonathan, and good morning, everyone. I'm happy to review Conexa's second quarter financial results and the highlights across our portfolio. Conexa continues to build upon the strength across our business, which is driven by both our commercial execution with ArchList and our pipeline development programs, including KPL387. ArchList continues to generate strong revenue growth. Our continued execution across key commercial drivers and increased penetration across the current pericarditis population led to a net revenue of $156.8 million in the second quarter. This represents a growth of $19 million over the first quarter. In just over four years since the launch of ArchList as the first and only FDA approved therapy for recurrent pericarditis, Conexa has generated over $1 billion in cumulative net sales. Surpassing this milestone is a result of our effective commercial strategy and our team who work relentlessly to bring this highly efficacious therapy to thousands of patients suffering from this debilitating disease. Conexa is well positioned to continue maximizing the potential of ArchList. For full year 2025, we're raising our ArchList net sales guidance to between $625 and $640 million from $590 to $605 million. Importantly, growing ArchList revenue continues to support our robust balance sheet, providing capacity for continued investment in value creating opportunities across the business without the need to access the capital markets. Turning to our pipeline, we've now initiated and have begun recruiting in the Phase II slash Phase III clinical trial of KPL387 and recurrent pericarditis.

This next slide

highlights the design of the Phase II, Phase III clinical trial. In designing this study, we've leveraged our experience with Rhapsody, which was the successful Phase III pivotal trial supporting FCA approval of ArchList in the current pericarditis. This study consists of three overlapping parts which have been combined into a single protocol the Phase II dose focusing portion, the Phase III double blind placebo controlled pivotal portion, and the long term extensions. We're now recruiting patients in the dose focusing portion of the study and expect data in the second half of next year. From there, we'll continue to move as fast as possible and our goal is to deliver this treatment option to patients in the 28-29 timeframe. Thanks to the excellent work of our teams, KINIXA is the leader in recurrent pericarditis. Importantly, we are committed to driving additional innovation for these patients and maintaining our leadership position. Our physician and patient market research shows an IL-1 alpha and beta inhibitor with the target profile of KPL387 could be a meaningful treatment option for patients with recurrent pericarditis. Specifically, the potential for a once monthly dosing of a liquid formulation in an auto-injector could drive further adoption as well as potentially enhance both duration and compliance. We continue to make solid progress in the second quarter both commercially and clinically and we continue to crack on across the portfolio. With that, I'll turn it over to Ross. Thank you, Saj.

Strong execution in Q2 led to significant revenue growth to $156.8 million, representing a 52% -over-year increase compared to Q2 of last year. This performance was driven by expansion in both the breadth and depth of the prescriber base, which led to the highest number of quarterly new patient enrollments since launch and resulted in a substantial increase to our active commercial patients. Additionally, we've seen good persistence from the Medicare Part D patients who transitioned to commercial therapy at the start of the year due to the affordability changes associated with the Inflation Reduction Act. We are seeing this patient cohort follow similar metrics to other groups of patients on ARCLIS and while the one-time bolus of patients observed in Q1 will not repeat, we have seen an increase in new Medicare Part D patients initiating commercial therapy versus the previous years. As a result of the increase in active patients, our penetration into the multiple recurrence population increased from approximately 13% at the end of last year to approximately 15% at the end of Q2. Ultimately, this growth reflects that patients and healthcare professionals continue to report high degrees of satisfaction with ARCLIS and we've built a robust foundation of commercial fundamentals. For example, in Q2, our payer approval rates remained greater than 90%, total duration of therapy was approximately 30 months on average, patient compliance with therapy remained strong at over 85% and we continue to see ARCLIS used earlier in the course of the disease. Importantly, our strong Q2 performance highlights the progress we've made, but more importantly, we continue to be even more excited about the significant opportunity ahead with ARCLIS. On this slide, I'm going to highlight how ARCLIS has continued to shift the treatment paradigm to become the standard of care for recurrent pericarditis. Our promotional efforts have been focused on educating patients and healthcare professionals to recognize recurrent pericarditis as an interleukin-1 alpha and beta mediated disease best managed with targeted immunomodulation. Since launch, we've seen continuous robust increases in both the new and repeat prescribers every single quarter. This growth not only speaks to the effectiveness of our educational efforts, but it also illustrates how receptive physicians have been to this evolved paradigm that utilizes a targeted, highly efficacious, and well tolerated treatment. In Q2, more than 325 additional healthcare professionals wrote their first ARCLIS prescription, representing one of the highest -on-quarter increases to date and bringing the total number of prescribers to more than 3,475. Additionally, repeat prescribing also continued to increase, with more than 120 ARCLIS prescribers writing for their second patients. Finally, we've also seen an increase in prescribing earlier in the disease. Of all the patients on ARCLIS, around 20% were prescribed ARCLIS while on their first recurrence, and roughly 80% when they had two or more recurrences. This highlights the growing physician appreciation for the value ARCLIS provides in preventing their patients from suffering future flares. In addition to more patients receiving ARCLIS at every stage of the disease, there has been a marked increase in the number of dedicated pericardial disease centers where patients are able to access expert care for healthcare providers, well-versed in their disease. We have sponsored the AHA's Addressing Recurrent Pericarditis Initiative as part of our ongoing efforts to shorten the treatment journey for patients by providing expert care close to home. There are also several more dedicated pericardial clinics outside of this initiative, and our aim is to continue supporting this growth to help patients gain an earlier diagnosis and appropriate treatment of their disease. As the treatment approach continues to change across the country, there's a growing body of published literature recommending IL-1 pathway inhibitors such as ARCLIS to be used ahead of corticosteroids, which is well aligned with our commercial positioning of ARCLIS. Furthermore, looking at data from Resonance, our real-world evidence disease registry, which is driven by expert pericardial centers across the country, ARCLIS has increasingly become the second-line treatment choice after NSAID and culture C. In Q2, we delivered $156.8 million in net revenue as well as increased the franchise profitability. As a result, we are pleased to increase our 2025 net revenue guidance by $35 million between the midpoints of the prior range and of the new range. This takes us from expecting between $590 to $605 million to now expecting between $625 and $640 million. This guidance indicates -on-year net revenue growth of $215 million at the midpoint compared to full year 2024. This would be the highest annual increase in net revenue to date. As you can hear, we're excited about the future of ARCLIS as well as the progress of our pipeline. We are determined to bring future launches of novel therapies to patients who are suffering from debilitating diseases. With that, I'll turn the call over to Mark to discuss our financial results.

Mark.

Thanks Ross. This morning I will cover our second quarter 2025 financial performance. You can find our detailed financial information in today's press release. There are a few items I'd like to call your attention to. First, starting with our income statement on the left-hand side of the slide. ARCLIS revenue grew 52% -over-year in the second quarter to $156.8 million, driven primarily by strong growth in new patient enrollments, prescribers, and active commercial patients. Operating expenses grew 26% -over-year in the second quarter, driven primarily by cost of goods sold and collaboration expenses from continued ARCLIS revenue growth and SG&A in support of ARCLIS commercialization. Lastly, due to strong revenue growth coupled with more moderate expense growth, net income was $17.8 million in the second quarter compared to a net loss of $3.9 million a year ago. Second, the right-hand side of the slide provides the calculation of ARCLIS collaboration profit, which grew 75% -over-year in the second quarter to $104.8 million, driven by sales volume and disciplined commercial investment. Third, at the bottom of the slide, our cash balance increased by approximately $40 million to $307.8 million in the second quarter, and we continue to expect our current operating plan to remain cash flow positive on an annual basis. As you've heard from Sanj and Ross, both commercial and clinical execution in the second quarter added to Canixa's significant momentum across its business. Combined with financial discipline and a strong balance sheet, Canixa remains well-positioned to continue to help patients as well as to create additional value in both the near and long term. And with that, I'll turn the call back to Sanj for closing remarks. Thanks, Mark. As you've heard,

Canixa continues to execute both clinically and commercially and is well positioned to build significant future value. We are dedicated to helping as many patients as possible with ARCLIS and to advancing the development of our clinical portfolio, which includes KPL387, the liquid formulation IL-1 receptor antagonist, which has a target profile of monthly dosing. Our ultimate goal is to bring additional treatment options and therapies to patients suffering from debilitating diseases with unmet need. I'll now turn the call back to the operator for questions. Thank

you. Certainly. And our first question for today comes from the line of Anupam Rama from JP Morgan. Your question, please.

Hey guys, thanks so much for taking the question and congrats on the quarter. I know you highlighted 15% penetration into the multiple recurrence setting at the end of 2Q. Wondering if you could provide some commentary on kind of the trends that you're seeing in the first recurrence setting. I think the slide said about 20% of total prescriptions are coming from this setting. Thanks so much.

Yeah, thanks very much, Anupam. This is Ross. Thank you for the question. So you're right that in the two-plus recurrence group, as a reminder, that's the 14,000 patient population in any given year. We've seen continuous increase since the launch into the penetration into that group. Most recently going to 15% versus last reported at the end of 2024 of 13%. So seeing some nice increase within that group and that remains our key target base as the patient groups who are suffering the most at the highest burden of the disease, also most closely aligned with the data in RAPTIDY as well. But we've also seen as the treatment paradigm has changed over time, significant growth in earlier on in the disease. So those patients are still very much within label, the broad label that we have just for recurrent pericarditis, agnostic to the number of flares. And there's an additional group of 26,000 patients in that first recurrence group, which is a significant opportunity for us. And I think what we're seeing now with around 20% of all the ARCLIS patients that were prescribed to the drug when they were on their first recurrence is greater confidence, familiarity, knowledge of how to prescribe, how to look after patients while they're on ARCLIS, and just greater comfort overall for healthcare professionals having greater experience with the drug and having seen the impact that it has on patients in the real world setting to utilize this drug earlier on in the disease. I think as well as an increase in understanding the recurrent pericarditis is a disease which is mediated by interleukin-1, alpha and beta. And in order to control that disease and prevent future flares, patients having to needlessly suffer those future flares, the utilization of an inhibitor of interleukin-1, alpha, beta, is being very well received by both patients and healthcare professionals. So we're pleased to see an increase across really the whole population, which is a total of 40,000 patients in totality when you include the first recurrence on top of the

two

plus recurrence

groups. Thanks so much for taking our question.

Thank you. And our next question comes from the line of Jeff Meacham from CIDI. Your question, please.

Hey, guys. Thanks for the question. I just want to follow up on Anupam's question. I guess when you look at the patients who've dropped off, maybe over say the past year or so, was the dosing frequency a big driver? I guess I'm trying to get a sense for what the new start outlook could be for 387. And I'm under the assumption that you'll have a pretty good switch rate as well looking from ARCLS. Thank you.

Thanks, Jeff. So we're seeing the patients continuing to stay on therapy for quite some time. We're seeing an average of 30 months in total. Patients are also pretty compliant to therapy overall at 85% or more. And patients are reacting very well while on ARCLS. So we're pleased about that. And we believe that there's a significant opportunity ahead for ARCLS as we continue to switch on more and more physicians. And when you take the penetration numbers of 15%, I think that shows that we've had good growth up until this moment in time. But the opportunity ahead is significant. And that's without taking into account the first recurrence patients. So we're very focused on continuing the growth of ARCLS. Maybe I'll pause there. Stan, do you want to comment on 387?

Yeah, no, thanks, Jeff. I mean, obviously we're very excited about 387. And we are definitely cracking on as far as the phase two, phase three studies concerned. Obviously that will be data dependent. But clearly we've shown we know how to commercialize in this space. We've developed an awful lot of great contacts and relationships with physicians. So that will be key. But ultimately dependent on data. But we certainly know how to do it. And as I said, we've leveraged a lot of the learnings we had from Rhapsody and from ARCLS earlier

on. So we'll continue to keep working on it. OK, thanks, guys.

Thank you. And our next question comes from the line of Paul Choi from Goldman Sachs. Your question, please.

Hi. Thanks, everyone. Good morning and congrats on the quarter. My first question is, just given the pace of growth here, how do you think about potentially further expansion of the sales force and or some sort of larger form of marketing slash DTC to continue to expand awareness and drive penetration? And my second question is, with 387, how are you thinking about potential in-office utilization in the future as part of the paradigm? Do you envision this potentially being more administrative in-office and just how you're thinking about self-administration versus physician administration down the road here? Thank you very much.

Thanks, Paul. This is Sam. Maybe I'll make a few comments and pass over to Ross if he has anything to add. But yes, very excited about the growth that we've had in ARCLS without a doubt. And as far as the sales force is concerned, obviously, as we've always said, we do an amazing job on what the right sizing is, looking at the territories. And we've done that, as you know, since launch just over four years ago. And as you've heard in the past, we have increased that. So at the moment, really, we've not made any comments as to exactly what we've done on the size of it. Last report was around 85. We're certainly continuing to look at what's needed as far as growth is concerned. But I think they're being utilized incredibly well. And as far as DTC and other things, clearly, our marketing groups had a great impact in launch so far. We certainly don't rest on our laurels. We're certainly looking at, in addition to what we've done as far as the sales force is concerned and our existing materials and disease education, physician education, we continue to look at other ways. I'm sure Ross can go into more detail. But we've certainly got a massive focus on digital marketing and looking at other ways we can really apply not just the metrics but also some of the new technologies that are out there to identify patients that are very much in need. So it's a very exciting area for us. As you can see, it's still growing. There's an awful lot more we can do. We certainly are tapping into that in the future. But maybe Ross, you can comment on how we're looking at marketing and expanding our assets there.

Yeah, absolutely. Thanks, Sanch and thank you, Paul. Yeah, I think the key thing here on top of what Sanch said is we are an organization that never rests on our laurels. We're quite happy with how the launch has gone to date. But we have so much more to do as an organization. And yeah, we've got to be very innovative in our approach, constantly evolving and refreshing what we do and finding better ways of doing things. As we get more and more into this market and increase our understanding of the market, we find that we can get more effective over time as we just have greater understanding. The utilization of newer technologies is also important to us. We have looked at utilizing AI in our targeting strategy and in a variety of different digital marketing environments. That's proven very successful for us. We haven't shared great detail on that. But we're utilizing a lot of new technologies now, which is really paying dividends and helping us to get out there to physicians and to patients and making a difference in this marketplace. So we constantly evolve and look at new ways of doing things to get better and better over time. To the second part of your question, Paul, regarding in-office versus outpatient use, the vast majority of our list is in-outpatients under pharmacy benefits. With KPL387, obviously, as Sanch said, everything is data-dependent. We see as we progress further, but with a target profile of monthly and in a liquid formulation with the potential to go to an auto-injector, that also plays nicely into where the patients are, which is ultimately not wanting to be in hospital suffering from this disease, but being treated appropriately and kept at home and preventing flares and preventing them going into hospital for the future. So whether that precipitates a change in in-office versus outpatients and patient administers in their own home is to be seen, but we don't see a substantial call in hospital utilization.

Okay, great. Thank you for taking our questions.

Thank you. And our next question comes from the line of Eva Fortea from Wells Fargo. Your question, please.

Good morning. Congrats on the quarter and thanks for taking our questions. Two quick ones from us. So on our call list from the 30-month average therapy duration, can you give us a sense in terms of numbers on how, you know, are you seeing a shorter treatment duration in the patients in first recurrence versus patients in second and beyond? And the second question is on 387. Can you discuss how you're thinking about like the balance between remaining cash flow positive on an annual basis and initiating studies in new indications beyond recurrent pericarditis? Thanks.

Thanks, Eva. Thank you very much for the question. So I'll certainly take the first part and then hand over to someone else to cover the second part. Yeah, we're not really seeing any meaningful differences in terms of duration from the different cohorts, including, you know, patients on a first recurrence versus second, third, fourth, fifth recurrence groups. But of course, you know, the data is always evolving and building and as more patients starting to be initiated on the call list earlier on in the disease, you know, we just don't have some of that data yet, but it will build and we'll report as we see it later down the line. But from what we see so far, no significant differences. The average is 30 months. The median of the initial duration of therapy is around 17 months and the read-start rates remains around 45%. I think importantly, of all those patients that started ARCLS in our launch quarter back in Q2 of 2021 now, around 10% of all of those patients that started way back then are still on therapy, meaning their initial treatment of therapy and have just stayed on, you know, throughout, which I think is testament to the effect and how well tolerated ARCLS can be. For many of these patients. So no significant differences today. It's obviously something that we will keep an eye on and report as we see.

And as far as your second question regarding cash flow and further investment, I think at this point in our life cycle, you know, we are focused on continuing to create value and importantly, as we've talked about in the past, you know, we do think that through commercial execution and continued financial discipline, we do have the capacity to continue to create value across our business, whether it's to further maximize the opportunity with ARCLS to further advance our pipeline and or to pursue

strategic initiatives. Got it.

Thanks. Thank you. And as a reminder, ladies and gentlemen, if you do have a question at this time, please press star one one on your telephone. Our next question comes from the line of Roger Song from Jefferies. Your question, please.

Great. Congrats for the call. And thank you for taking our question. Maybe two quick ones for the pipeline. The first one, KPL 387. So understanding you are doing the phase two portion to decide the dosing. Just curious about what what is the target Africa safety profile, particularly in the context of comparison to our artists. You can make decisions to decide those and how likely you will multiple those into the phase three portion, something like induction maintenance. And then a quick one for the 1161 and the enabling right now. And then what's the current thinking about potential indication for this quarterly? I want thank you.

Thanks for that question. We were actually both those questions. Regarding the profile of KPL 3, 7, what we're looking for in the dose focusing portion of the trial is to select the dose that we'll use in the pivotal portion of the trial. And so in that sense, the ability to treat the acute flare as it happens and then prevent the subsequent flare is really the profile that we established with our list. And that we're looking for similar profile, if you will, with regard to the KPL 3, 7, efficacy. So once we selected that dose, we then carry that forward into the randomized withdrawal portion, pivotal section of the trial, which bears remarkable similarity in terms of its design, in terms of the endpoints that we have structured. And so at that point, it will be data driven in terms of what the actual profile of KPL 3, 7 is. But we're very confident in the study design as being able to show the strength of KPL 3, 7 and its target profile of one monthly dose. Regarding the second question of 1161, at this point, we've not announced any specific indication. We realize that there's broad potential of having an IL-1 alpha, IL-1 beta inhibitor pathway inhibition, if you will, that has a target profile of dosing every three months. And so that really opens the possibility of a range of chronic lifelong diseases that are auto-inflammatory. So we'll continue to do that work. But in the meantime, our focus is on the IND-enabling studies so that we can begin the first in-human study as soon as possible. Thank you so much. Thank you.

Thank you. And our next question comes from the line of David Nearinggarden from Wedbush Securities. Your question, please.

Thanks for taking the question. I had two and maybe one is kind of a follow-up to the last one. But is there any specific kind of efficacy boundaries that you're looking for out of the 387 study? So, of course, ARCLA showed a near 100% drop off in recurrences. Is that the kind of efficacy bar we should be thinking about or could it be a little bit lower because of the more convenient dosing? And then I had another question on just emerging competition. There's a potential oral competitor out there that's going to report out data. This half, is there any thinking on that or any thoughts on how we should think about the potential competition emerging?

Thanks. Thanks, David. Appreciate the questions. So, regarding the efficacy profile in the phase two studies, the dose focusing portion, we're really looking across a range of different dose levels in order to understand the performance characteristics of KPL387. And so, in that sense, we intend to use the totality of the data in order to define the dose level that we'll take forward. So, at this point, it's a little early to describe exactly what the expectation is precisely, but rather to say that I think this will be a very informative study based upon its design that will help us optimize the performance of the drug. Regarding competition that's on the horizon, we remain the leaders in the space of recurrent pericarditis for the reason that we have really done the deep work in understanding the mechanism of this disease. And we've identified the fact that IL-1 alpha and IL-1 beta inhibition is a critical element for maintaining control of the disease and important fact to be able to maintain control of the disease as monotherapy. And so, we continue to look with interest to see other data as they emerge, but understanding those, shall we say, those mechanisms will have to define themselves in the context of the fact that, as I mentioned, we understand that complete control of the disease requires control of both. Thank you.

Thank you. And this does conclude the question and answer session of today's program. I'd like to hand the program back to Sanj Patel for any further remarks.

Thank you, Operator, and thanks everybody for the questions and joining the call today. We look forward to the remainder of the year and to providing additional opportunities and updates in the future. So, very excited and thank you very much.

Thank you, ladies and gentlemen, for your participation in today's conference. This does conclude the program. You may now disconnect. Good day.