Kiniksa Pharmaceuticals International, plc Class A Ordinary Shares Wells Fargo 21st Annual Healthcare Conference

Thank you very much. Very happy to be here, Eva.

Certainly happy to. Thank you very much, Eva, and thanks to everyone in the room for joining us and everyone online for listening in. We are very happy to be here, and thank you for the hospitality, and the meetings that we have set up today. John and I here from Kiniksa, we will be making some forward-looking statements, which are subject to risks and uncertainties, a full copy of which can be found in our SEC filings. Maybe if I start with a high-level overview of the organization, and then we can dive into whichever part of the organization you would like to, Eva. Kiniksa is a well-capitalized, growth-orientated organization. We have been around for around 10 years now as a company. We have been a commercial stage organization for just over 5 years, with ARCALYST in recurrent pericarditis. ARCALYST is doing very well commercially.

In Q2, we announced a net revenue of around $243 million, which is one of the largest quarter-on-quarter growths, in fact, the largest quarter-on-quarter growth that we've had launched to date. We feel like we're still relatively nascent in that market, with a substantial opportunity left to help more and more patients. We're very pleased with how ARCALYST is going, and we increased our net revenue guidance for 2026, for the full year, to between $980 million and $995 million. Behind the commercial asset, we also have a pipeline of drugs, which are progressing rapidly, at different stages into the clinic and through the clinic. We have KPL-387, which is being investigated in recurrent pericarditis. We announced just a couple of months ago, some of the phase II data, for that, which is the dose-focusing portion of our total studies.

We're pleased to say that we have moved forward with the target product profile into the phase III, which is the monthly subcutaneous drug, which will hopefully be in an auto-injector formulation, in recurrent pericarditis. Not only did we announce that data around the phase II, but also that we have initiated the phase III study and already dosing and enrolling patients in the phase III study. Behind that, we also have KPL-1161, which is an Fc-modified drug, which could go into different indications that are IL-1 mediated, of which there are many. This is potentially a quarterly drug, which again is focused on the inhibition of IL-1α and β. The organization is well capitalized. We have around $525 million in cash reserves, at the end of Q2, and have historically been profitable. We're very excited about the future.

With that, maybe we can dive into the questions, Eva.

Yeah, it's a great question. We certainly think that there's a lot of growth opportunity left within this marketplace, and there's several ways of looking at that opportunity, whether it's by the number of patients or the number of prescribers, and we can go into some of those metrics. Ultimately, we feel like we're relatively nascent and making good progress. There's a lot to do in this marketplace. I think one by one, we're making progress, and we're switching on more and more physicians to this new way of prescribing and treating patients with recurrent pericarditis. The literature is also being populated with the new paradigm of how to prescribe and how to treat this disease, which we're very pleased with. Ultimately, as we've announced before, we're around 21% penetrated into the population that's two or more recurrences.

You'll know, if you've been following Kiniksa for a while, that the recurrent pericarditis population is around 40,000 patients, and that's the indication for ARCALYST. If you subdivide that into the number of flares a patient has suffered, and you look at those that are in two or more recurrences, that's a 14,000 population out of the 40,000. We're about 21% penetrated at the end of 2020, sorry, at the end of Q2 of this year, into that population of the 14,000. That's without even accounting for the larger number of patients that are on the first recurrence and within label, which is an additional 26,000 patients. I think that tells us that, to get to 21%, we've been making good progress.

Clearly, there's an awful lot more to do within the two-plus recurrence group, and we're getting growing utilization in the first recurrence group as well, and physicians ultimately making the decision that they don't have to wait for their patients to suffer future flares before treating them earlier on in the disease with targeted interleukin-1 alpha and beta inhibition.

I think there's a lot of things. It's kind of multifactorial, all the things that we're focused on as an organization and how we've been evolving this marketplace. Some of those things are, as I mentioned earlier, around the population of data in publications. We've always been promoting ARCALYST since the time of launch, 5 and a bit years ago, as a therapy which is steroid-sparing, which is one of the things that physicians used to reach towards to treat this disease, really through lack of other treatment alternatives. The population of now or the populated data, is really showing that physicians should be using interleukin-1 alpha and beta prior to corticosteroids. So after the utilization of NSAIDs and colchicine, prior to corticosteroids, to opt for interleukin-1 alpha and beta inhibition.

That's how we've been promoting it since the time of launch, and is aligned with our data. As I think more physicians kind of understand that and get the messaging through dissemination of the data, and particularly through the ACC Concise Clinical Guidance, which was published midway through last year, in August of last year, which affirms that type of treatment paradigm. I think more and more physicians are coming on board with that and recognizing the new way of treating the disease. So certainly publications and just experience in the marketplace of this evolving treatment paradigm are certainly helping. Obviously, our field execution continues to be incredibly important.