Good morning or good afternoon, ladies and gentlemen. Welcome to our First Quarter 2023 Results Call. And before I go into the call, I would like to have the next slide and show you the forward-looking statement slide, as we will be making some forward-looking statements that are based upon our future expectations and our current expectations and assumptions. And as you know, they may change in the future. And without – having said that, next slide, please. I'm here with my three colleagues, Anurag Relan, our Chief Medical Officer; Stephen Toor, our Chief Commercial Officer; and Jeroen Wakkerman, our Chief Financial Officer. And they will be speaking after me. And I will start with a brief introduction. So next slide, please. And then the next one as well. So basically what Pharming is all about is that we are building a sustainable rare business and that rare business will be able to be funded from the positive cash flows that we continue to generate from RUCONEST. And you have seen over the last quarters that these cash flows from RUCONEST have helped us to prepare and fund and the launch of Joenja in the United States. And the further pipeline development that we have been doing. So we're in a very favorable position and today marks the first quarter in which we have been able to actually get Joenja approved and where in the last quarter where we will be reporting on sales from our single product. Because going forward, as you have seen from the press release, we will start a recording sales for Joenja in United States as well. So it's an important demarcation, I would say, in the history of our company that we are now going to work to get revenues from two products, albeit…

Thanks, Sijmen. Before we look at the launch of Joenja, let's review a little bit about APDS. We can see that on the next slide that APDS is a rare primary immune deficiency, also known as an inborn error of immunity that was only first characterized just a little over 10 years ago in 2013. We estimate that it affects approximately 1,500 patients in some of the countries that you see listed there, and that's based on a prevalence of 1.5 per million. To-date, we have already identified more than 500 of these patients across these areas. The treatment options until recently have been quite limited for APDS patients, we'll talk a little bit about that, but this is really, the treatment has been limited because it's only been focused on the symptoms of the disease. These symptoms begin early in childhood, but do not address the root cause of APDS. And the signs and symptoms vary and we'll talk a little bit about what those look like, but they vary even within a family. And this results in significant delays and especially due to the delayed onset of the diagnosis. The diagnosis itself is actually quite simple to make when the clinician thinks of it, and that's really through this genetic test that can provide a definitive diagnosis. Next slide. But as we see here, APDS really impacts patients in many ways. There's of course, the physical manifestations and you see that really in the top left with the recurrent infections that these patients have, the enlarged lymph nodes and glands. They have numerous infections that lead to damage in their lungs and a whole slew of symptoms there that you can see due to this progressive serious disease that develops early in childhood. On top of the physical…

Thank you, Anurag. Good morning, everybody. Over the next four slides, I’m going to provide you with an overview of the Q1 RUCONEST performance and some early insights as to the progress of the Joenja launch just six weeks after approval. As you’re aware, there were HAE market-wide issues that impacted some government insurance patients, resulting in a delayed product shipments. Our internal data and external audit data showed significant declines for all acute prophylactic products, and – sorry, all acute and prophylactic products, and that impacted all companies serving HAE patients. The issues are resolved late in the quarter, and as effective patients started shipping RUCONEST sales accelerated, and the product staged the highest positive return or bounce back of all the acute products in the market. With the Q1 market-wide issues and disruption behind us, we saw good sales in March and also strong sales in April as the recovery continued. And we also, of course, as the slide alludes to see strength in the underlying business, more especially high volumes of new patient enrollments and growth in prescribing physicians. We therefore expect sales to strengthen through Q2, and we continue to forecast low single digit revenue growth for RUCONEST in 2023. So now let’s turn to the Joenja launch. As can be seen in this slide, Pharming’s bring in all of its rare disease commercialization experience to bear in the U.S. Our first of many launches on a must win market. We have 54 salespeople and sales leaders, and that’s comprised of the RUCONEST sales team, where we think 30% of patients are treated by customers already very well served by Pharming and the new Joenja institutional team. And this team focuses on central locations or centers of excellence to which we expect the other 70% of…

Yes, thank you very much, Steve, and good morning, good afternoon. As – next slide please, as Steve mentioned earlier, as you can see on this slide, the first quarter results were lower. And that was due to the HAE market factors, which impacted the entire industry. And those industry-wide factors have since resolved, and we can confirm that we have strongly recovered. And if we look at the quarter of January was in line with last year. And February is where we faced headwinds. March had a strong recovery, and so had April. We’ve almost made up all of the shortfall and expect to recover the remainder. We therefore continue to expect single digit growth in RUCONEST revenues for 2023. And on the slide, you see that the revenues in Q1 were $42.5 million. That’s 9% down on last year for the reasons I mentioned. Gross profit developed in line with that. It went down by 8% to $38.5 million, and the operating cost increased from $40 million to almost $53 million. And that was on the back of leniolisib and R&D investments and in sales and marketing costs. Operating profit and net profit reduced. And the operating loss was $13.7 million and a net loss $12.2 million. So the short of this quarter is that the seals shifted from Q1 to Q2, and we’ve seen that in April. And with regards to cost, we’re investing in leniolisib and obviously we haven’t recorded any revenues in Q1 yet for leniolisib, Joenja. But that will change in Q2. We then go to the next slide, please. On the cost development, you see that we are continuing to invest in a launch of Joenja. If you look at the longer-term trends over the quarters that are shown here, starting with the…

Thank you. [Operator Instructions] Our first question comes from Alistair Campbell of RBC. Alistair, your line is now open. Please go ahead.

Thanks very much. Yes, a couple of questions, please. First of all on RUCONEST [Technical Difficulty] understand what’s going on here. Is this a feature of basically sales which would’ve happened? Or is there actually genuinely a shortfall in sales? Just trying to understand, if that had – if the disruption hadn’t happened, would you be more likely to get maybe something like mid-single [Technical Difficulty] single digits? Question two is, I know it’s very, very early, but just to get a bit of insight into the patients you’ve put on to Joenja. At this stage, do you have sort of a sense of the severity of those patients they sort of across the spectrum of severity, or do they tend to come year end? And if I can push malarkey, you’ve got 23 patients on therapy now, what do you think a good number would be to be sort of exiting [Technical Difficulty]?

You were breaking up your third question…

Sorry, third question was, given you’ve got 23 patients on Joenja now, what do you think would be a good exit number for the end of the year?

Okay, thanks. Let me answer the last one and I’ll go back to Stephen on the first two questions. We don’t give as you can appreciate, its early days and we don’t give any sort of forward-looking statements on what we think is a reasonable number. I think it’s too early. But you obviously agree with us that already having 23 patients on paid therapy and many more in the enrollment process gives you a good indication that we prepared to launch very carefully. Also Stephen talking about how well we are progressing with getting the reimbursement sorted for Joenja. So let’s keep it at this. And obviously over the coming quarters we will continue to report on those patient numbers. And going forward, we will give some more indications as and when we see a clear trend arising. So that’s the answer to question number three. I’m sorry I can’t go any further detail. And I would like to go back to Stephen about your question with regards to RUCONEST sales in the first quarter and the – if there’s any sort of differentiation in the severity of your Joenja patients. Steve, open to you.

Thank you, Sijmen. In terms of the RUCONEST sales, I really think our guidance wouldn’t have changed. There was disruption in the first quarter, which pushed patients out a little in terms of delivery of product. So at this point, we’re playing catch up. So I think the guidance would remain the same as it was in mid to late March when we last gave it, which is expect low single-digit revenue growth. In terms of severity of patients, I don't have deep insights. What I would hypothesize and perhaps invite Anurag if he's got anything to add, is that these are patients largely already identified. At least half of them were in EAP or in the open-label extension. So they were identified, therefore they were exhibiting symptoms and were at least at the moderate end of the scale. Does that answer the question?

Yes, that's great. Thank you.

Sorry, I was just going to…

Go ahead, Anurag.

I think the key point is that there was a mix of patients here. So we had patients who were in the study that have now started Joenja commercial paid product. We have patients who were in the expanded access program, and we also have naïve patients. So these are patients who were not in the expanded access program or in the study who are now receiving Joenja, leniolisib for the first time.

Okay. Does that answer your question?

That’s good, thank you.

All right, thank you.

Our next question comes from Sushila Hernandez from VLK. Sushila, your line is now open. Please go ahead.

Yes. Thank you for taking my question. On RUCONEST, could you expand on the reimbursement disruptions affecting the HAE market? Since Takeda showed an increase in TAKHZYRO sales this quarter, what were the circumstances that led to a more pronounced disruption on your end? And a second question, you mentioned that you expect quarterly fluctuations from RUCONEST sales, what are the drivers behind these fluctuations that you're anticipating? Thank you.

Yes, Sushila, I’ll hand these questions back to Stephen.

Certainly, I mean, the disruption was primarily in the government sector, and some of those patients saw disruptions to their copays and the cost of accessing medication which is what resolved itself as we went through the quarter. I can't comment to the company, to the sales obviously, of other companies. What I will say is if you look at Symphony data, for example, on their, specifically their Metys database, you see that every company saw significant disruption in Q1. So I can't comment to that, but I can say that the disruption was specifically within the government insured patient sector, and it resolved satisfactorily. And all those patients certainly on the RUCONEST side are now receiving their medications.

And the other question, Sushila?

Yes, certainly, you mentioned that you expect quarterly fluctuations of RUCONEST sales. So what are these drivers behind these fluctuations that you anticipate?

Apologies I missed that. So it tends to be obviously more a combination of seasonal and just the length of the selling month. So for example, we tend to see some dips during the significant U.S. holidays, so Independence Day, Thanksgiving, around Christmas. So it's not – and also during the holiday season as well, where patients will stock in ahead of going on vacation and then not order as much during the periods when they're away. And I think it's the fact we're not driven by the law of large numbers, right. We have a certain amount of patients, and when the change in their order and patterns changes, then you see changes in our order rates and therefore quarterly fluctuations. So there's nothing of real significance beyond that.

Okay. Thank you.

Thank you.

[Operator Instructions]

Mr. Smedes [ph], no more questions.

Our next question comes from Simon Scholes of First Berlin. Simon, your line is now open. Please go ahead.

Yes. Hello. I see you’ve taken the decision to discontinue Pompe. I remember a few years ago you expected your product under development to have quite a benign side effect profile compared with the current market leader. I was wondering if you could comment on your decision to discontinue given your expectation of that positive side effect profile. And also whether you took the decision to discontinue, maybe because it would’ve taken too long to bring the product to market if that played a role?

Yes, it’s a good question, Simon. I think there’s still a significant unmet medical need in Pompe. So that’s not necessarily the case. We did not simply see the differentiating features that we felt confident enough to go forward with investing in the project. So therefore we basically decided to stop and abandon this project. And there’s also, of course, new developments on the horizon where other than protein replacement therapies, for instance, the GYS1 receptor antagonist are being developed as we speak. So we thought it was appropriate to stop this bearing, but not seeing any of the differentiating features. That was the main reason to make the decision.

Okay. Thanks very much.

Any more questions, Simon?

[Operator Instructions] And there are no further questions. I’ll hand back to the management team for any closing remarks.

Okay. Thank you very much. Ladies and gentlemen, thanks for attending this first quarter results conference. As I was saying in the beginning of the call, this first quarter marks a clear demarcation. We got our second product approved here. We have established commercialization infrastructure in the U.S. and we’re building that up in Europe. So we’re preparing rigorously for the launch of many outside the U.S. I hope you agree that we are off to a very good launch with regards to Joenja in the United States because of the fact that we already have these 23 patients on therapy within six weeks after launch, which is not given in rare diseases and many more are already in the process. So we look forward – to very much forward, I would say, to coming back to you next quarter and report on not one, but two products that will drive our revenue. And of course, last but not least, we remain confident. I would like to again say that we remain confident in the robustness of our RUCONEST business going forward. So thank you very much for being here again. And we look forward to updating you again on the next quarter results in the beginning of August. Thank you. Goodbye.