Kiniksa Pharmaceuticals International, plc Class A Ordinary Shares Canaccord Genuity's 46th Annual Growth Conference

Certainly will do. Thank you very much, Edward, and thank you to the Canaccord team for inviting us here today. It's a pleasure to be here. And thank you to everyone in the room, as well as online that's listening. John and I will be making some forward-looking statements today, which are subject to risks and uncertainties, a copy of which can be found in our SEC filings. Sanj Patel, our CEO, is not here today. Unfortunately, he could not be here, as him and his wife Kristen welcomed a new baby into the world just a couple of days ago. So it's John and I today, and hopefully we can answer all the questions that you have. So thank you very much for having us. Many of you may be familiar with Kiniksa at this point.

We've been on the market for the last five years or so with ARCALYST. The company is about 10 years old overall. Really, it's been quite an interesting story over the last 10 years or so. We're an organization that likes to move rapidly, and we are very focused on growth and adding value across the years and the pipeline that we have. We are a well-capitalized organization that's been profitable, and we've said that we intend to be cash flow positive on an annual basis moving forward. We have ARCALYST on the market over the last five and a little bit years, which has been growing very well in recurrent pericarditis, and ARCALYST is an interleukin-1 alpha and beta inhibitor with quite impressive both efficacy and safety profile. That's been doing very well in the market since the time of launch, with very significant opportunity ahead.

In Q2, our earnings call just a couple of weeks ago, we announced the net revenue for the quarter was around $243 million for Q2. That was a $29 million quarter-on-quarter growth, which was the largest quarter growth that we've had since the time of launch five years ago. As I said, the opportunity is still very substantial for what we can achieve within that marketplace with ARCALYST. The pipeline is also very strong. At Kiniksa, we have the KPL-387, which is another interleukin-1 alpha and beta inhibitor. We announced some of the phase II results, which were the dose-focusing portion of our overall studies in recurrent pericarditis just a few weeks ago. We can go through that data throughout the meeting today.

We essentially announced that we were moving forward into the phase III development with the target product profile that we specified right up front, which is a monthly, potentially auto-injector formulation for an interleukin-1 alpha and beta. We are now moving forward into the phase III, and in fact, we have started, initiated the phase III study and are already dosing and enrolling patients, and we expect to be on the market, data willing, by the 2028, 2029 timeframe. We also have behind that KPL-1161, which is an Fc-modified interleukin-1 alpha and beta inhibitor with a potentially quarterly profile, which could have utility in multiple disease areas. The company is well-funded, well-capitalized, and very focused on growth for the years ahead.

Thank you. Edward, maybe I will make a start on that. John, if you have things to add, then you can. Maybe if I just kind of ground us all in the size of the population for recurrent pericarditis. This is really a very severe, very debilitating disease for many patients. There are around 160,000 patients in any given year that suffer from pericarditis. Many of those patients just suffer as it is kind of a once and done type of incidence of pericarditis, albeit very debilitating during the time of that initial flare. Unfortunately, a proportion of those patients go on and suffer from recurrences over time, and that is where recurrent pericarditis comes in, and that is around 40,000 patients in any given year.

That can then also be further subdivided between the number of flares that patients suffer, but ultimately, there is a 40,000 patient population in any given year that are suffering from recurrent pericarditis. Unfortunately, many of these patients, being a rare disease and a flaring disease, which is also very widely dispersed around the country, there is a lack of real centers of excellence per se across the country that are looking after this disease. Patients are very widely dispersed, being seen by many cardiologists, many rheumatologists. Unfortunately, what happens with many of these patients is that they get diagnosed later on in the disease, as well as going through misdiagnosis along the way as well. In fact, there is around 2.7 on average misdiagnoses before the patients get diagnosed with recurrent pericarditis.

Increasing the education and awareness on how to identify this disease and obviously treat the disease now that there is an approved therapy on the market to specifically address the underlying mechanism of interleukin-1 alpha and beta of the disease. It is obviously very important for these patients to get an earlier and a timely diagnosis. That is generally the patient population, and what you may know is the treatment paradigm has changed quite significantly over time, whereas patients used to be treated often on the very first incidence actually with NSAIDs and colchicine, and then after that, moving forward to steroids, ultimately corticosteroid use, through having really a lack of other treatment options to try to control the disease. That has been changing very substantially over the last five years or so since the introduction of ARCALYST as the first and only approved therapy.

We have seen lots of publications and guidance, for example, from the ACC, that have also been affirming that change in treatment paradigm, which is ultimately to opt for an interleukin-1 alpha beta inhibitor after NSAIDs and colchicine and prior to corticosteroid use.

It certainly makes up a sizable portion, Edward. What we have said previously is that we are focused on the entirety of the 40,000 patient population. That's where we have the broad label for ARCALYST, and the label is completely agnostic to the number of flares a patient must have suffered before they get access to the only approved treatment. The data that we have provided externally is the penetration rate, as you said, Edward, into the 2+ recurrence group, which is a 14,000 patient group of the 40,000 overall. Clearly there are an even larger group of patients on the first recurrence, 26,000. We've said that we've been growing over time, and that we've been up to around 21% as of the end of Q2 penetration into the 14,000 patient group. Those with 2 or more recurrences.

We also have seen growing utilization in the first recurrence as well, and physicians ultimately getting much greater comfort of how to prescribe a biologic and how to manage patients when they're on ARCALYST. Ultimately, taking the view that along with the ACC concise clinical guidance, as I mentioned as well, is ultimately why allow patients to suffer for more flares throughout their disease when there is a treatment option there to help them. The importance, and what we learn a lot from patients, is not only wanting to quickly, rapidly overcome the flare that they are often suffering at the time of prescription of ARCALYST. But the most important thing to patients is preventing future flares for the future. Ultimately having the knowledge that this is, once a patient becomes recurrent pericarditis, this is usually a multi-year chronic disease for most patients.