Thank you very much, Adam. Good morning or good afternoon, ladies and gentlemen. Welcome to our Second Quarter and First Half 2023 Financial Results Call. Next slide, please. I’m here with my three colleagues, Dr. Anurag Relan, our Chief Medical Officer; Stephen Toor, our Chief Commercial Officer; and Jeroen Wakkerman, our Chief Financial Officer, to take you through the highlights of these results and, of course, to answer all your questions that you may have afterwards. But before I do that, of course, I would like to point you to the next slide that says something about forward-looking statements because we will be making some forward-looking statements, of course, today, that are based upon our current plans, belief, market circumstances, et cetera, that may, of course, change towards the future. And then without further ado, I would like to move on to 2 slides ahead and basically share with you some of the excitement over the last six months that we had, of course, and we had a very busy six months and indeed made a lot of progress. And – but first and foremost, we’re, of course, very, very pleased to see that RUCONEST did it again, so to say, 20% growth over the second quarter results versus the first quarter is, of course, a very spectacular recovery from what was a one-off weakness in the first quarter. And that means that if you look at it, we’re very confident that we can actually continue to be on track for the low single-digit growth for the entire year. And we say that based upon the leading indicators that all point in the right direction. Of course, Stephen, will talk a little bit more about that later in his part of the presentation. Now that significant cash flows, of course,…

Thanks, Sijmen. And on the next slide, we can see a little bit of information about APDS, which is a rare, serious and progressive primary immune deficiency, and, as you heard from Sijmen, just first described about 10 years ago. We think that APDS affects about 1.5 million – 1.5 patients per million based on literature estimates and our own patient finding efforts. And I’ll talk a little bit about those patient-finding efforts in a moment. But with those efforts, we’ve identified now more than 640 patients in key global markets already. We think that, that represents a portion of the total of 1,500 patients or so that are out there with APDS. The signs and symptoms of APDS vary widely, and as with many rare diseases, there’s also a significant delay to diagnosis because of these varying symptoms and the rarity of the disease. These delays result in significant morbidity and mortality for these patients. And it’s something that we’re really trying to address with our own patient-finding efforts and making Joenja available, of course. The treatments for APDS up till now have been focuse on symptom management. So really, when we think about the problems that these patients face, so with infections, so giving them antibiotics or giving them immunoglobulin replacement therapy, really not addressing the root cause, however. A genetic test, however, is a very simple way to make the diagnosis of APDS, and we’ll talk a little bit about some of the things that we’re doing on this front, too, in a moment. On the next slide, we can see that Joenja was now approved by FDA earlier this year. Joenja modulates this hyperactive pathway and as a result, allows the immune system now to develop properly. This development of the immune system when it occurs…

Thanks, Anurag. Good morning, and good afternoon, everybody. I’m going to provide you with a short update on RUCONEST progress in Q2 in the first half and also the Joenja launch in Q2 of this year. Next slide, please. So as Sijmen touched upon already, the disruptions we saw in the first quarter were HAE market-wide, and they also affected our competitors. And as I indicated in the Q1 call, they were transitory. In the second quarter, RUCONEST performed well. The leading revenue indicators, including growth in unique prescribers, new patient enrollments and vials shipped to patients were all strong. In fact, new enrollments have hit 70-plus for both Q1 and Q2 despite the Q1 market issues. And this really clearly reflects the underlying demand in the HAE market and for RUCONEST specifically. In the second quarter, RUCONEST revenues globally increased by 20% compared to the first quarter. And furthermore, we saw a 2% increase in revenues when comparing the second quarters of 2023 and 2022. So given the strong bounce back in the second quarter, which we did signal in the Q1 call, we’re maintaining our outlook of low single-digit revenue growth for the rest of 2023. Next slide, please. This slide shows the number of unique prescribers continues to grow in the U.S., 687 represents 62% of the HAE prescribing community and is still growing nine years post-launch, as Sijmen mentioned earlier. And that’s despite the significant changes to the market throughout those years. This clearly shows that despite the prophylactic launches and the genericization of FIRAZYR, there remains both a place and need in the treatment armamentarium for a C1-esterase-inhibitor and an enduring need for RUCONEST. We expect to continue growing the unique prescriber base in the coming months and years and with, of course, the base…

Yes. Thank you very much, Steve. Good morning, good afternoon, everybody. Moving to the next slide on the financial highlights for the second quarter of ‘22. Our revenues grew to $54.9 million, which is a growth of 9% versus last year. And the $54.9 million consists of $3.8 million of Joenja sales in the first quarter that we obviously report that Joenja sales, $51.1 million from RUCONEST. And that is a growth of 2% from last year’s second quarter, but 20% even from Q1. So a very strong recovery, as Sijmen and Steve mentioned before as well from Q1. Gross profit grew to $49.2 million, which is plus 7%, which is roughly in line with the growth in revenues. Operating costs increased by $23.4 million, and that’s on a number of items, mostly marketing and sales, but I’ll give you some more detail later. Also important to understand for the second quarter is that we had in what we call other income, so that’s between gross profit and operating costs in the P&L, we had an income of $21.1 million for the sale of the priority review voucher to Novartis, which was as per the agreed contract in 2019. And also good maybe to tell you that in the last year’s second quarter, we also had some big other income that was connected to the BioConnection transaction, as you may remember, which brought us in $12.8 million. To cut a long story short, the increase in other income from this year to last year was $8 million. Then on the operating profit, $5.3 million. That’s a decline and that’s roughly in line with the increase in the operating costs and the net profit went from $15.7 million to $1.3 million, so a decline of $14.4 million. And that is, by…

Thank you. [Operator Instructions] And our first question comes from Christian Glennie from Stifel. Christian, your line is open. Please go ahead.

Yes. Good afternoon, guys. Thanks for taking the question. I guess we’ll start with Joenja. And just initially on – just to explain or sort of clarify the 60 patients on enrollment versus the 43 on paid therapy, is it the case that some of those are – they’re on therapy, the other 17 are on therapy already, but still working through in terms of reimbursement? Or how should we think about those – the balance of those 17?

Christian, when patients actually are enrolled, they get a free starter pack, which is a one-month supply of Joenja. So yes, once they are enrolled, they have access to the medication, so they have it and then, of course, the administrative procedures to get them reimbursed starts. And if that happens within the months, the second month will be a commercial pack. And if that procedure is not yet finished then there will be another month’s supply in the form of a bridging pack provided. So to answer your question, yes, they’re all having access to therapy once they enroll. I hope that’s clear to you.

Yes. That’s good. Thanks. And then in terms of the patient – identified patient numbers, you’ve added 140 in terms of 640 versus the 500 you previously reported. I noticed that the U.S. has stayed the same. Just where is those extra 140 patients come from? Is that mostly these new international markets, Canada, Australia, Japan or where are they from?

Could you comment on that, Anurag? Is that possible?

Sure. Hi, Christian. So we are finding patients – we’re continuing to find patients in the U.S., but we’re also finding patients in other markets. So that does include Japan, Australia and other markets where we intend to commercialize first.

Okay. Thank you. And then in terms of the – you’ve got 200 patients in the U.S. identified, if you do your 1.5 per million in the U.S. there’s potentially about 500 or so out there. That’s already a 40% diagnosis rate. Where do you think that could – that diagnosis rate could go over the next few years in terms of identifying patients?

Would you like to answer that as well, Anurag?

Sure. So we definitely see this trending up. I think when we think about markets in Europe and some other countries across the world where there are more centralized health care systems, we can see that the prevalence there already exceeds 1 per million. In the U.S., of course, we have a more distributed and less centralized system. So the diagnosis of these patients is – and the care for these patients is a little bit more fragmented. And so we expect that, over time, that diagnosis rate will go up as there’s increasing awareness of the disease, but that there’s also an available therapy available for these patients. So I think that we expect that to increase at the minimum approach what we see in some other countries, but likely increase beyond that. And that’s why we think we’re being reasonable about an estimate of 1.5 per million. As I mentioned earlier, there is a significant population of patients that we found beyond the 200 in the U.S., for example, that have these variants of uncertain significance or what are termed to be VUS. And I think that, that will – that’s another pool of patients who have symptoms of a primary immune deficiency, have had a genetic test, oftentimes, that’s well before we’ve been involved in the picture even, but they still don’t have a clear diagnosis. And I think this will likely lead to an increase in the number of diagnosed patients over time.

Sorry, as a quick follow-up to that then, is it your understanding that those patients would also be appropriate for Joenja even if they haven’t got sort of formally defined genetic mutations that are driving APDS currently, or understood to drive it?

Yes. That’s correct. They would be appropriate for Joenja because they would have APDS if that’s confirmed. Right now, they have a – oftentimes, they have symptoms and clinical manifestations of APDS, and they have variants or mutations in one of the two genes that leads to APDS. The question is, are those mutations, are those variants disease causing? And that’s part of the effort that we’re helping clinicians gather information, get access to further testing, and help them resolve that question for those patients because they’re also, like I said earlier, sitting in limbo and don’t have a clear diagnosis yet. If they get a clear diagnosis of APDS, then those patients would be potentially eligible for treatment with Joenja.

Thank you. One final one, if I can, and I’ll get back in the queue. Is there any possibility to give any rough sort of guidance for Joenja for the year, whether that’s in terms of patient numbers or revenues? Obviously, you’ve got 60 patients in the program already, $3.8 million revenue in the second quarter. That 60 patients is already a 40% penetration of the 150 patients in the U.S. that are over 12. So any sense for penetration rates or patient numbers by the end of the year would be great.

Yeah, I understand that, Christian, but it’s early days, right? And yes, we’re off to a good start. And we would like to see a bit more development of numbers. Obviously, the numbers are ticking up all the time, right? And we’ll keep you updated on a quarterly basis for now until such time that we get a little bit more feel for the market and the development going forward. And then we will start giving some different guidance. So for now, no, not yet. You have to be a little bit more patient for that. But we will eventually, of course, do that. But we’ll keep you, on a quarterly basis, updated on the number of patients that are on therapy and are enrolled, okay?

Yes. Appreciate it. Thank you.

Thank you.

The next question comes from Joe Pantginis from H.C. Wainwright. Sir, your line is open. Please go ahead.

Hey, guys. Thanks for all the details. I appreciate it. So a couple of questions on the initial dynamics of the Joenja launch. So I guess – and of course, prefacing it by, this is all early still, what is the general time it takes or approximate time it takes to get drugs to patients once they’re identified? And then second, when you look at these numbers, the 60 and the 43, how many patients have gotten the drug or are on the drug, of the revenue number that you posted today, which, like you said, beat overall expectations, how much of that, if any, is based on just getting drugs into the inventory channel? Thanks.

Okay, that’s an excellent question, probably for Steve to go into a little bit more detail. Steve, could you pick that one?

Yes. Sure. Thank you so much. Good morning, Joe. Sure. So the first thing is, as Sijmen alluded to, once the patient’s enrolled, we give the starter pack out, and so they’re on therapy pretty quickly. And then in terms of the time it takes to get drug – sorry, to get approval for patients, it’s typically, we’ve seen so far between four and six weeks, which is pretty quick. We’ve seen one or two is a little quicker or one or two is a little more, but think of it in a four to six-week period. So we’re not having to bridge too many patients beyond the starter pack. And then in terms of stocking, it’s almost zero. We put some in right at the very start. We were able to process patients very quickly after launch, which means we burned through that very quickly. And the way in which our partner works, PANTHERx, means we’re basically practicing just-in-time delivery. So for the most part, that revenue is generated by demand – by patient demand.

That’s great to hear. Thanks. And then, oh, yes, absolutely. So I feel I have to ask my next obligatory question that I usually go to. When you look at OTL-105, so obviously, you’re holding it close to your chest, but I’m curious, with the ongoing preclinical models that you’re doing, will we be able to see or when we’ll be able to see any of the data coming out of these models?

Would you like to comment on that, Anurag?

Sure. Good morning, Joe. So we’re making progress together with Orchard in those models. I don’t have a firm timeline that I can say that it’s going to – this is going to progress in the next couple of weeks or not. But I can say that I expect probably toward – later this year, we should be able to provide some further updates on where we are with that program, the data that those preclinical models are generating, and how we plan to move the program forward.

Great. I appreciate the details, and nice to see the strong launch on Joenja.

Thank you.

The next question is from Sushila Hernandez from VLK. Sushila, your line is open. Please go ahead.

Yes, Thank you for taking my questions. Also on Joenja, so you mentioned it takes four to six weeks to get approval, but could you also elaborate a bit more on the time from identification to enrollment? Could you share a bit more on that? Thank you.

Steve, would you like to comment on that?

Yes. Thanks. So it’s obviously variable, but relatively quick once a physician and the patient have that discussion, it’s literally governed by the time it takes to complete the form, send it into the hub, and then generate the starter pack. So I mean it’s a day to a week, but really dependent on the speed at which that process happens. But it can be very, very quick, easily a day or two.

Okay, that’s clear. And just on RUCONEST, you mentioned that over 70 quarterly new patients. Where are these patients coming from? Are these treatment-naive patients or did they switch?

For the most part, these will be switch patients. At this point in the evolution of the market, there are very few treatment-naive patients. So these are patients who are either not satisfied on their current acute therapy, or require, as per the guidelines, a second acute therapy to make sure that they have what they need to do with any breakthrough attacks.

That’s clear. Thank you. And then a final question, could you share progress on your BD activities? How is it progressing?

That’s a good one, Sushila. We have a very active team. I think if I sort of remember some numbers, they turned over 150 opportunities over the last 12 months. There were – the vast majority, of course, was quickly disposed off. But then we have an internal community, including Anurag and Stephen, who look and take a next look. And then, out of that, a much smaller number, of course, comes to the fore, and we do some more analysis on that. And we’ve done a few due diligences over this last year – just last year as well. So we’re making – I think, I can say the efficiency of the BD process has significantly improved over the last year. And we came, a couple of times, very close, but we’re very careful, of course, with the first one. And as you know, in business development, it’s all or nothing, right? There’s no deal or there’s a deal. So – but yes, we remain optimistic about being able to acquire or in-license, preferably in-license, of course, that’s the preferred mode of action, and another asset that is in mid to late-stage development so that we can actually plan for another asset to be launched in the not-too-distant future. And that’s all I can say at the moment, I’m afraid.

Okay. Looking forward to updates on that front as well.

Thank you.

[Operator Instructions] And the next question comes from Natalia Webster from RBC. Natalia, your line is open. Please go ahead.

Hi there. Thank you for taking my questions. And I just have two, one on RUCONEST and one on the leniolisib. So on RUCONEST, we saw an improvement in Q2 and you reiterated your guidance for the full year. But I was wondering if there’s any risk of the reimbursement issues that we saw in Q1 reoccurring at all. And then also you mentioned sort of strong market demand and continued growth in unique prescribers. But I was wondering if you’re able to provide some color specifically around what you’re expecting for H2 and sort of in the near-term into 2024. And then secondly, on leniolisib – or do you want to go to that first? I can ask the next one after?

Yes, probably a good idea to let Stephen answer that question first, you’re okay?

So firstly, the event that happened in Q1, we’re not expecting a repeat of that. It was a very specific market-wide event that was unrelated to Pharming. It affected everybody and it was rectified towards the end of Q1. My suspicion is that’s very much a one-time event and I don’t expect that to occur again in the future. And then in terms of growth in prescribers, it’s actually been very consistent for the last seven or eight years, certainly I’ve been with Pharming, and we retook control of RUCONEST. There’s still, as I mentioned, another 38% of HAE-specific prescribers in the U.S. who’ve not yet tried RUCONEST and we continue to call on them as well as our core customers. So at this point, I see no reason, given the last seven or eight years, to not expect that same steady growth as we move forward.

Great. Thank you. And then just secondly on leniolisib, regarding the timing for the CHMP decision, you say that you expect the same queue for this year, but I think you said H2 previously, so just wondering if there’s been any delay there or you’re just being a bit more specific.

Do you want to be answering that, Anurag?

Yes, I think we’re just continuing to work collaboratively with the CHMP and EMA in answering their questions. And I think we’re just giving some more detail as we have more detail around the timeline there, and that’s why we are saying the fourth quarter now.

Thank you.

Next question comes from Hartaj Singh from Oppenheimer. Hartaj, your line is open. Please go ahead.

Hey, this is a [indiscernible] on for Hartaj. Congratulations on the good quarter for RUCONEST and the nice start for Joenja. I have questions on the APDS patients. How can we think about the growth rate of APDS patient number quarter by quarter and would seasonality be a factor to consider? Thanks.

So you ask if there’s any seasonality in APDS, is that right?

Yes. I mean, the growth, like, how can we think of the growth rate of APDS patient number quarter by quarter and would seasonality be a factor to consider down the road?

Steve, you’d like to comment on that?

Thank you, Sijmen. I mean, obviously it’s an ultra rare disease. We’ve had a bonus of patients to start with, but we’re still through the – we’re still in the – very much in the launch phase. So I’d expect steady growth as we move forward and really no seasonality, but obviously in the outer years, you’ll start to see the rate of growth slow as you would do with any launch, but nothing specific in terms of seasonality that we – where we would expect significant drops or spikes. Does that answer the question?

Yes. Very helpful. Thank you.

We have a follow-up from Christian Glennie from Stifel. Christian, your line is open. Please go ahead.

Hi. Thanks. Just I thought it’s worth pulling up on, obviously, you filed applications in Canada, Australia, Israel, potential approvals next year. What’s the commercial strategy there? Is it better to find partners? Is it worth doing it yourself in some of these markets? What should we expect there? And obviously in Japan, you need to run a trial, that’s a bit further down the track. What would be the strategy?

Yes, yes. First of all, Christian, before I hand over to Stephen, there’s no trial requirements, right, in Australia and in Canada. We have submitted the files. We have received a priority review as well from those authorities. So it’s a matter of waiting for, first of all, the validation of the files – regulatory files. And then of course, we’ll go through that and then we’ll await the opinion of those regulatory authorities somewhere, as alluded to in someone, probably mid first half of next year. Then I’ll hand over to Stephen to explain how we are approaching the commercialization in those markets.

Thank you, Sijmen. Hi, Christian. So certainly, I think to answer the heart of the question, we have no plans to out-license in any of those markets and give away, frankly, the value of the product to other companies. The structure, though, in the go-to-market with just Joenja would be different from say the U.S. or Europe where we have pretty large footprint. So it would be a light cost-efficient footprint. And in somewhere, for example, like Australia, you would service that market directly with probably a hybrid model, a combination of directly employed Pharming employees, and then local distributors and partners who can help us service certain elements of the Australian infrastructure. But to keep things really efficient from that market, you would also serve Hong Kong, South Korea, which is the third biggest market in Asia Pac, and other smaller markets. So the plan is very much for us to keep control of the product, keep control of bringing patients on therapy and obviously, reaping the benefits of that for ourselves and our stakeholders. Does that answer the question, Christian?

Yes, that’s very clear. Thank you.

Thank you.

[Operator Instructions] As we have no further questions, I’ll hand back to the management team for any concluding remarks.

Thank you very much. Thank you, ladies and gentlemen, for attending our conference. And I would like to remind you of some of the elements of the outlook for the remainder of the year. You’ve heard all the positive developments on the leading indicators, the forward-looking indicators, underpinning the sales of RUCONEST, that we remain confident to continue to guide on the low single-digit growth for RUCONEST revenues for this year. You’ve heard about the steady flow of new patients coming in for Joenja and, of course, the immediately available patients that could be brought to Joenja therapy over the coming quarters. You’ve heard about the regulatory interactions that we have with the Europeans, the plans to follow-up in the United Kingdom and the submissions, of course, to broaden our footprint with the submissions in Canada and Australia. And, of course, that we are continuing to invest in the launch preparations in those markets and, of course, the clinical trial plans that we have for the second indications of leniolisib, which, of course, we will update you as and when we have received the feedback from the regulator on our plans that have been submitted to the regulators with regards to both the – what the new indication is and that indication, by the way, as you already have learned from us, it has a bigger patient potential than APDS. And we will refill that later on during the year. And last but not least, we hope to be able to come back to you during the remainder of the year, of course, with in-licensing or acquisition news, because we are very keen and have the capabilities, of course, to further leverage our commercialization infrastructure that we have and that we are basically expanding towards the coming years, including markets like Australia, Canada, as you heard from Stephen, but also Japan, and to bring more products to that market to further significantly accelerate the growth of our company going forward. So thank you again for being at our conference. And we look forward to updating you on the next quarter results somewhere in the last week of October. Thank you very much. Goodbye.