Kiniksa Pharmaceuticals International, plc

Thank you for standing by and welcome to the Connexa Pharmaceuticals Second Quarter 2026 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 the session, you'll need to press star 11 on your telephone. If your question has been answered and you'd like to remove yourself from the queue, simply press star 11 again. As a reminder, today's program is being recorded. And now I'd like to introduce your host for today's program, Jonathan Kirshenbaum, Investor Relations. Please go ahead, sir.

Thank you, operator. Good morning, everyone, and welcome to the Canixa Pharmaceuticals second quarter 2026 earnings call. A press release highlighting our financial results and recent portfolio execution can be found on our website under the Investors section. As for the agenda, our Chief Executive Officer, Sanj K. Patel, will start with an introduction that will be followed by Ross Moat, our Chief Operating Officer, who will provide an update on Arculus commercial execution. From there, Connexa's Chief Medical Officer, Dr. John Paolini, will review our KPL387 development program and the ongoing Phase 2-3 clinical trial in recurrent pericarditis. After that, Mark Ragosa, our Chief Financial Officer, will review our second quarter 2026 financial results. And finally, Sanj will share closing remarks and kick off the Q&A session. 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 such statements. A review of these 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 as the date of this presentation, and we undertake no obligation to update such statements except as required by law. With that, I'll turn it over to Sanj.

Thanks, Jonathan, and good morning or good afternoon, everyone. Connexa is in a strong position more than halfway through 2026 as we continue to execute across our portfolio. We continue to make excellent progress with ARTLIST and have advanced the KPL 307 program into the pivotal stage with the initiation of the phase three trial, which we have named PASTORAL. Additionally, KPL1161, which is our FC-modified IL-1 alpha and beta inhibitor with a target profile of causally dosing, is progressing well, and the program remains on track to initiate a phase one study by the end of this year. Importantly, we've continued to maintain a robust financial position, which together with strong commercial momentum and key advancements in our development pipeline positions the company with multiple value creating drivers in both the near and long-term. Our ongoing execution of the Onculus commercial strategy resulted in a meaningful increase in the number of patients on therapy. Robust revenue growth of more than $29 million over the previous quarter draw sales of $243.6 million in the second quarter. And we continue to build on our strong commercial momentum, and we've raised our full year 2026 revenue guidance from $930 to $945 million to between $980 and $995 million. On the clinical side, just this morning, we announced data from the dose-focusing portion of the KPL387 Phase 2, Phase 3 study in recurrent pericarditis. On the basis of the Phase 2 data, I'm happy to report that we are moving forward with the target profile of a monthly dose into the pivotal Phase 3 portion of the trial. Today, we also announced that this Phase 3 study has already started and is now enrolling and dosing patients. The ongoing Phase 3 study, Passerall, marks a key milestone in bringing additional treatment options to patients suffering from recurrent pericarditis. This trial follows Rapsody, the successful Phase 3 program with Arclist, with both trials having the same registrational endpoint of reduction in the risk of pericarditis recurrence. John will share additional details about the phase two data in a moment, as well as provide an overview of the design of this phase three study. We anticipate a potential commercial launch of KPL 307 in the 2028 and 2029 timeframe. Extending our leadership in the recurrent pericarditis market so we can help many more patients. And with that, I'll turn it over to Ross to review our commercial execution. Ross.

Thank you, Sanj. In Q2, the Conixa commercial team continued to drive strong growth with Arclist. Our net revenue was $243.6 million which is more than $85 million growth versus Q2 of 2025 and more than $29 million growth compared to Q1 2026. This represents the largest quarterly net revenue increase since our launch more than five years ago and is a direct result of the execution of the strategy that we laid out at the beginning of this year. Our commercial approach has helped to change the treatment paradigm for recurrent pericarditis, and we are focused on continuing to unlock future growth for Arclist. Over time, we have made disciplined, value-driven investments across our sales infrastructure as well as innovative strategies that have enabled us to reach more patients. Firstly, we've been diligently ensuring that prescribers have a positive prescribing experience, which encourages deeper prescribing as well as peer-to-peer education to other healthcare professionals. Additionally, we've been investing in machine learning and the use of AI to provide our sales team with insights on not just who to target, but importantly, when to visit and what messages should be delivered. Secondly, in April of this year, we launched our targeted DTC campaign called Hearts Home. This campaign is aimed at educating and empowering patients who are suffering from recurrent pericarditis to visit their healthcare professional and ask for Arclist. So far, the campaign has reached thousands of patients who are suffering from recurrent pericarditis. While it's still in the early stages, We are starting to see encouraging signs of engagement with our campaign and patients visiting their healthcare professional to discuss Arclist. Thirdly, our teams have been highly focused on disseminating the 2025 ACC Concise Clinical Guidance for Recurrent Pericarditis. The understanding of this guidance and the recommendation of moving Arclist earlier in line After NSAIDs and colchazine and ahead of corticosteroids has helped to expand the utilization of Arclist. Since our focus on disseminating this publication, we've seen an increase in doctors who have changed their treatment approach and are now using Arclist earlier in the disease course. Finally, the commercialization is underpinned by Arclist and many more. As of the end of Q2, our penetration into the multiple recurrence population had grown to approximately 21% compared to around 18% at the end of 2025. This demonstrates both strong growth as well as the substantial opportunity ahead. As mentioned on the last slide, we're seeing continued momentum in the breadth and depth of ARCALIS prescribing, which in Q2 led to significantly higher number of new patient enrollments compared to any quarter since our launch. Approximately 450 additional healthcare professionals wrote their first ARCLIS prescription in the second quarter, bringing the total prescriber base to more than 5,000 launched to date. As a reminder, there are more than 25,000 healthcare professionals across the country who manage recurrent pericarditis patients. Therefore, the opportunity for continued growth is evident. Additionally, the number of healthcare professionals who have written multiple prescriptions increased by approximately 150 compared to Q1 of 2026, meaning that around 29% of the prescriber base have written ARCLIS for two or more patients. The dual acceleration in both new and repeat prescribing led to an increase in patient enrollment. which resulted in a substantial increase to the number of patients on therapy and illustrates the demand for a highly efficacious treatment that protects patients from suffering unnecessary additional flares. As you can hear, we're pleased with the Q2 performance, but we continue to be even more excited by the opportunity that's ahead. And with that, I'll turn the call over to Dr. John Paolini to share more information on our KPL 387 program in recurrent pericarditis.

John. Thank you, Ross. As Sanj mentioned, the pivotal phase three trial of KPL 387 in recurring pericarditis has now been initiated and is already enrolling and dosing patients. Before that, I'll provide A brief overview of the Phase 2 dose focusing portion of the trial, data from which affirmed the dose level for Phase 3. As a reminder, for the Phase 2-3 study, we have combined both portions into a single integrated protocol in order to maximize operational efficiency, thus allowing the Phase 3 portion to begin even while the Phase 2 trial is still ongoing. Phase 2 is designed to define the PK-PD relationship and provide information on the cadence and magnitude of initial response, as well as the durability of effect of defined subcutaneously administered KPL387 dose levels. Up to approximately 80 participants presenting at screening with a pericarditis recurrence despite treatment with NSAIDs and colchicine are randomized equally into four arms to receive subcutaneously administered KPL387 at 100 milligrams or 300 milligrams dosed biweekly or once monthly. Concomitant treatment with conventional oral therapies is weaned and discontinued within two weeks to attain KPL387 monotherapy. The primary endpoint of the phase two dose focusing study is time to treatment response through 24 weeks, defined as an NRS score of less than or equal to two on the 11-point daily pericarditis NRS pain scale, and normalization of C-reactive protein, a marker of pericardial inflammation. The data we announced today show that the KPL387 300 milligram monthly dose level demonstrated rapid and sustained onset of action with durable efficacy throughout the monthly dosing interval, affirming the 300 milligram monthly dose being evaluated in the phase three study, Pastoral. Specifically, in this analysis, median time to treatment response was four days with a 95% confidence interval of three to six days. Median time to pain response was four days with a confidence interval of three to six days. And median time to CRP normalization was eight days with a confidence interval of seven to nine days. The cadence and magnitude of these reductions in pain and inflammation are consistent with prior studies which supported ARCA list approval in recurring pericarditis. KPL387 was generally well tolerated consistent with the well-known safety profile of IL-1 pathway inhibition. Regarding the other dose levels in the study not selected for phase 3, the 100 mg subcutaneous bi-weekly and monthly dose levels showed some effect but not at the level to support further study. The KTL387 300mg bi-weekly dose level was efficacious, without incremental benefit above the monthly dose level. The totality of data available supported the initiation of Pasteural with the 300mg subcutaneous monthly dose level. The Pasteural design should look familiar based upon our prior work in Rhapsody. This pivotal phase 3 trial is a placebo-controlled, event-driven randomized withdrawal study designed to measure the reduction in risk of pericarditis recurrence as the primary demonstration of KPL387 efficacy for the label. The primary efficacy endpoint is time to first adjudicate pericarditis recurrence during the randomized withdrawal period. The trial will enroll up to approximately 85 participants experiencing a pericarditis recurrence despite conventional oral therapies into a single blind run-in period during which KPL-387 is initiated and oral therapies are weaned and discontinued. Participants are blinded to the duration of the run-in period. Subsequently, participants who respond to KPL-387 in the run-in period then enter the randomized withdrawal period in which they either continue receiving kpl 387 300 milligrams once monthly or switch to placebo upon closure of the randomized withdrawal period participants may be eligible to continue into a long-term extension as we have mentioned our goal is to bring this potential new additional treatment option to patients in the 2028 I will now turn the call over to Mark to cover our second quarter financials. Mark?

Thanks, John. This morning I'll walk through our second quarter 2026 financial performance and highlight the key drivers behind the results. As always, detailed financial information is available in today's press release. The quarter reflected strong execution with continued momentum across our commercial business Advancement of our development pipeline and further strengthening of our financial position. Starting on the left hand side of this slide with the income statement, ARCLIS revenue grew 55% year over year to $243.6 million in the second quarter. As you've heard from Ross, this growth was driven by continued expansion in new and repeat prescribers as well as patient enrollments. Operating expense growth year over year was driven by several factors. higher costs of goods sold due to ARCLIST revenue growth, increased collaboration expenses aligned with higher ARCLIST revenue and collaboration profit, higher R&D primarily due to the increased KPL387 clinical trial costs as well as manufacturing costs, and also increased preclinical development investment. And lastly, additional SG&A primarily driven by investment associated with the commercialization of ARCLIST. Together, these factors contributed to year-over-year increases in operating income and net income, which were $27.2 million and $25.4 million, respectively. The calculation for ARCLOS collaboration profit, which drives total collaboration expenses, is on the right-hand side of the slide. Here, we continue to leverage disciplined commercial investment as ARCLOS collaboration profit grew faster than sales on a year-over-year basis. increasing 68% to $176.1 million. Turning next to cash, at the bottom of the slide, we ended the second quarter with a $525.9 million cash balance, representing approximately $58 million of net cash generation for the period. Looking ahead, we believe our operating plan enables us to continue helping patients while creating additional value over both the near and longer term. With that, I'll turn the call back to Sanj for closing remarks.

Thanks, Mark. As you've heard, Connexta is well-positioned to build significant future value as we grow our IL-1 Alpha and Beta inhibition franchise. We are dedicated to helping as many patients as possible with ARCLIS and to advancing the development of our clinical portfolio in order to bring additional therapies to patients. With that, I'll now turn the call back to the operator for questions.

Certainly. And once again, if you have a question at this time, please press star 11 on your telephone. Our first question comes from the line of Nick LaRusso from TD Cowan. Your question, please.

Thanks very much for taking our question and congrats on the very strong quarter, guys. So as you guys mentioned, this was the strongest quarter of Arculus absolute sales growth since launch. So I just wanted to drive into what drove this growth specifically in Q2, and could this level of growth continue throughout the rest of the year and into next year? Thanks.

Thanks, Nick. I'll say a few comments, and I'm sure I'll leave it to Ross to dive into some detail. But look, as always, it's continuing to execute across the entire commercial strategy. And overall, growing the adoption of IL-1 pathway inhibition as the preferred treatment for recurrent pericarditis. And that continues to happen every quarter. Certainly this quarter, Russell bowed into the number of new unique prescribers we've had this quarter, as well as the number of new repeat prescribers and the new enrollments. But ultimately, it's a matter of just continuing to penetrate into the total population, which we're doing. And obviously the total duration of therapy is very important. That's about in line really with the median duration of the disease, about three years. but ultimately penetrating into that as we just reported we're now around 21 percent penetrated into the multiple recurrence population but that to me represents a meaningful opportunity ahead and so that's what we're focused on so very excited about it. Ross, why don't you dive into some of the drivers and the actual numbers across those metrics but there's special sources just continuing to execute.

Thanks Sanj and thanks for the question Nick and I think that's absolutely right I mean this is a culmination of a lot of work across all our commercial and cross-functional teams at this point and where we've got to really understanding the recurrent pericarditis market and really just generally executing flawlessly across the board. So really kudos to all of the team that have been able to help so many patients throughout Q2. Ultimately, we've seen a substantial uplift in the number of both new prescribers and new repeat prescribers. As Sanj said, we have more than 450 new prescribers coming to the total prescribing base in Q2, more than 150 new repeat prescribers, meaning they're prescribed for two or more patients in the quarter. and ultimately that's kind of grown the number of patients that are on therapy which led to both the results in Q2 being the highest number of incremental revenue that we've had in any quarter since launch as well as the highest number of new prescribers. Ultimately some of the driving factors underneath that as well as some of the actions that we've put into place around investing in AI and machine learning trying to make our field team even more effective than they historically have been knowing just not only kind of who to call upon in a very traditional targeting approach but more importantly now seeing when to call upon doctors and some of that's through claims analysis alerts but importantly predictive alerts as well of where patients may be coming into FLARE and visiting particular healthcare professionals As you know, we've also invested in the DTC campaign and that's starting to show some early signs of success driving patients into the clinic to ask specifically for Arclist. And I think the third point that's worth mentioning is around the dissemination of the ATC concise clinical guidance, which actually was published in August of last year. But generally speaking, because these recurrent pericarditis patients are very and many more. You know, aisle one inhibition is now placed after NSAIDs and colchazine use and prior to corticosteroids has been a key part in the transformation of really changing the treatment paradigm and being able to help many more patients. So I think all said, it's really, you know, rolled up to just ongoing solid execution across the team and acknowledging that, you know, even now, five years plus into the launch, there remains very significant opportunity for ARCLIS moving forward.

Thank you very much.

It's all very helpful. Thank you. And our next question comes from the line of Eva Forte from Wells Fargo. Your question, please.

Hey, good morning. Thanks for taking our question and congrats on the quarter. Two quick ones from us. So on your prepared remarks, you mentioned a higher number of new patient enrollment for ARCALIS this quarter. Is there a specific patient profile that you're seeing coming at this stage of the launch? The follow-up was, can you comment on the growth to net for the quarter? Thanks.

Ross, why don't you start with the big impact on the market? You can comment on the GTN.

Yeah, absolutely. Let's do that. Thank you, Eben, for the question. We haven't really seen any changes in the actual patient profiles as such, whether that's through demographics of the patients to what level we know about that, or the type of institutions or healthcare professionals that are prescribing. It's still really across the board, academic centers, more rural centers. The opportunity, I think, is still very broad across the country. So we haven't really seen a change in Thank you very much. as you know historically the misdiagnosis and underdiagnosis rate is pretty substantial and patients go through seeing many healthcare professionals before getting the recurrent pericarditis diagnosis so we've seen you know that I think improve over time and I think that's really really everything that is there's no no major changes and just happy to see more patients getting the help that they really need and deserve

David, your second question regarding gross to net in the quarter. Historically, gross to net does move lower sequentially in the second quarter. That was the case again this year. Year to date, gross to net is 7.2% down from 8.6% in the first quarter, with the main driver being lower copay due to the changes that we made to our support program at the beginning of the year. And I think as you look throughout the rest of the year here, we don't provide specific gross to net guidance, but we do anticipate co-pay support to continue to be favorable to gross to net on an annual basis with the majority of the impact having taken place in the first quarter. And additionally, we do expect sort of the normal pattern to hold here. So, absent any prior period reserve adjustments. You know, our gross net historically has been highest in the first quarter, a lower in Q2 and Q3, and then, you know, works a little bit higher in the fourth quarter as industry dynamics begin to play a factor. So I'll leave it there.

Very helpful. Thanks.

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

Oh, great. Hey, guys. Thanks for the question. Congrats on the data and the quarter. Just have a couple. So the first on 387, you know, now that you have the phase two data in hand and with commercial knowledge of the market, is there anything you guys have embedded in the phase three, you know, perhaps to further differentiate the profile of 387? And then second question, I know I usually ask, but I wanted to check on demand trends from the first recurrence population for ARCA labs. Is there maybe some element of that driving this quarter? Thank you.

I'm not sure if you want to come up on the Phase 3 study, but essentially, obviously, we're pretty excited about the Phase 2 results we've seen. We're obviously now moving into Phase 3. As we've said, we're excited about the target profile of KPO387, potential for monthly dosing, liquid formulation. That in and of itself, I think, is very exciting. But obviously, the data will be the data from the Phase 3, so we're just focusing on really getting through the rest of the enrollment on the Phase 3 study and hopefully seeing the results and being on the market in the 28-29 time frame. But John, any comments from the Phase 3 study?

No, I would say that the profile, as Sanj mentioned, of KTL387 that we're taking into the Phase 3 study is one that we're very excited about. Rapid onset of action, durable efficacy throughout the monthly dosing interval that's being studied in Phase 3 and passed around. So that sets us up in an excellent position for the pivotal Phase 3 trial. The design of the Phase 2 trial is one that we understand well and that is well understood in the scientific community as a randomized withdrawal study design. And so we are prepared to execute on that and to bring the trial forward.

Thanks, Jeff. I appreciate your question. So if I just answer your question on Arclist. Thank you very much. 80% of all new patient prescribing happening in the 2 plus recurrence so that's about 80% of new prescriptions that are coming in in a quarter for patients that are on their second or more recurrence and that's a 14,000 patient population and based upon that population that's where we mentioned that we're now penetrated around 21% into that opportunity and that's not accounting for the patients to your question directly that are on their first recurrence and Again, fitting within the label, that's a larger patient group. It's around 26,000 of the 40,000 patients. And we see about 20% of the Arclist prescriptions in Q2 that are within that patient group. And that's been growing over time. And I think some of that reflects the acknowledgement of the broad label, the increasing confidence of using Arclist, and ultimately the ongoing opportunity that there is ahead. Thank you.

Thank you. Thank you. And our next question comes in line of Nupam Rama from JP Morgan. Your question, please.

Hey, guys. Thanks so much for taking the question, and congrats on all the progress.

Just wanted to follow up on Big Meech's question here. On KPL387 300 mcdose, when I look at sort of time to response, pain, CRP, and compared to sort of the Rhapsody New England paper, the run-in period for ARCLIST, it looks very in-line-ish, plus or minus a day or so. Is that a fair assessment?

And can you remind us what your market research suggests a monthly regimen could mean commercially? Thanks so much.

John, why don't you start and then Ross, you can jump in.

Sounds great. Thank you, Anupam, for the question. Yes, we would concur that the data that we have shown from the Phase 2 trial in terms of Time-to-treatment response, time-to-pain response, and time-to-CO3 normalization, you know, are very robust and are consistent with what has been seen previously in trials that supported, you know, Rolanocephalic ruble and recurring parent carnitis, especially when one takes a confidence interval approach, you know, to looking at the numbers with your point estimates. Ross?

Yeah, thank you, Anupam. Yes, we did previously share some market research around Thoughts from patients and healthcare professionals on the target product profile of KPL387 versus kind of current commercial and other investigational therapies. And really that showed that, you know, both to a high degree for both the patients and the healthcare professionals, they were pretty excited about the KPL387 target product profile with around 75% of patients. say that they would prefer the KPL387 target product profile over current commercial or available or investigational therapies and around 92% of healthcare professionals indicating a high likelihood to prescribe for new patients in the context of the target product profile. Additionally, the potential availability of another IL-1 therapy could also expand the pool of patients for IL-1 inhibition overall with the monthly target product profile.

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

Hi. Thank you. Good morning, and thanks for taking our questions, and congrats on the quarter and progress. My first question is on the commercial side, and I was just curious if you're thinking about adding incremental headcount to your sales force at this point, just given the commercial momentum, or is the plan to continue to leverage AI and the advancement of the ACC clinical guidelines? My second question is on KPL387, specifically the transition study for patients who are stable. Can you maybe highlight what you're trying to show there and how you think what data are needed to support a potential switch strategy from Arculus down the road? Thank you.

Maybe I'll chime in, John, if you can take the rest. Thanks, Paul. So look, we're always looking at our Salesforce analytics. working out the best way to reach these physicians and healthcare professionals. That's an ongoing basis for us. So I don't think there's anything to report really on that side. As Ross mentioned, we continue to leverage the AI and machine learning and digital marketing, which has been really helpful. But again, it's just really one part. There are a multitude of ways that we've got to continue to keep working to continue to penetrate into the total population, which we're doing. So it's really might have no one-size-fits-all. You've got to continue to execute across all those functions. So we'll continue to crack on. Clearly, the 21% penetration so far tells you there's an awful lot more work to do, and that's what we're geared up to do. So we'll do it with the best where we can across all those different areas and hopefully continue to report information to you going forward.

Thank you, Paul, for your question. So, yes, the KTL387 transition to monotherapy were all involved in this dosing administration study. It was a phase two study designed to provide supplemental information for the label and to assist, provide basically the information to assist clinicians as they move across different therapies, if you will, for recurrent pericarditis. And so that trial is designed with different dosing regimens to test that efficacy and safety Regimens of NSAIDs in colchicine or corticosteroids or IL-1 pathway inhibitors, including anakinra and rolanacept. And so at the end of that study, with these different dosing paradigms having been tested, that enables the writing, if you will, of the dosing and administration section of the label in order to allow patients to move smoothly across therapeutic lines.

Okay, great. Thank you. Thank you, and our next question comes from the line of David Nerdingarten from Wedbush. Your question, please.

Hey, thanks for taking the question. I have one on 37. I have maybe two. First, just what was the kind of median follow-up? Was it the full six months for these patients or something else? And then, you know, if you saw any recurrences in the population in the study in the 300 milligram arms or any of the other ones, actually. Thanks.

Yeah, no, thank you, David, for the question. So the data that were obtained for this analysis is an interval analysis of the ongoing study. And as such, the disclosure is limited. And so what we can say is that we have harvested this information to affirm the 300 milligram monthly dose, certainly beyond the monthly dose and interval in order to show that at the trough, if you will, of the monthly dose and interval, the treatment effect is robust. and other than that, that is the limit of what we have said. What we have also said, though, is that the 300 milligram biweekly dose level, which of course delivers more drug, did not provide incremental benefit above the 300 milligram monthly dose.

Okay.

Okay, thanks.

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

Thank you, operator. Well, we better crack on. Thank you for all the questions today. Joining the call, we look forward to the remainder of the year and to providing additional updates in the future. Thank you.

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