argenx SE

Thank you. A press release was issued earlier today with our half-year 2020 financial results and second quarter business update. This can be found on our website along with the presentation for today's webcast. Before we begin, I'd like to remind you on slide two that forward-looking statements may be presented during this call. These may include statements about our future expectations, clinical development, regulatory timelines, the potential success of our product candidates, financial projections, and upcoming milestones. Actual results may differ materially from those indicated by these statements. Arjenx is not under any obligation to update statements regarding the future or to conform those statements in relation to actual results unless required by law. I'm joined on the call today by Tim Van Hauwermeiren, Chief Executive Officer, Keith Woods, Chief Operating Officer, and Erica Saldi, Chief Financial Officer. On slide three, you can see our agenda. Tim will be highlighting recent milestones, including the positive phase through the ADAPT data we reported in May, and the progress we've made in our ongoing EFGAR-TIGMAB programs and additional indications. We announced a change in our development plan for Kuznetsov this morning, and Tim will update you on the reason for that shift in strategy. And he will close with an update on our earlier stage programs, including those that are wholly owned and partnered. Keith will then provide an update on our commercial preparation, and Eric will share our financial results. It will then close with a Q&A session. I will now turn the call over to Tim.

Thank you, Beth, and welcome, everyone. We appreciate you joining the call today. We're now almost six months into what we can only continue to describe as unprecedented times. I hope you and your families are staying safe and healthy. At ArjenX, we have been opening our GAN offices on a restricted basis as we watch the data on the number of coronavirus cases in Belgium. We continue to keep our work from home mandate in the US and Japan. Throughout this virtual work environment, Our team has kept to its high work standards to minimize disruptions to our business as best as possible. For this, I must share my gratitude to the entire team in navigating this new normal. On slide four, I would like to highlight some of the significant milestones we have achieved virtually as a team, all of which will be covered in more detail later in the call. We executed a key phase C data disclosure with the top line readout of our ADAPT trial, including a subsequent financing to support our first commercial launch and the advancement of our differentiated pipeline. The data from ADAPT showed that Avgat Digimod has a promising therapeutic profile with robust efficacy and tolerability and the potential to offer individualized dosing to patients. These results, along with our having enrolled ADAPT ahead of pace, have further strengthened our leadership position among SGM antagonists. We have also worked hard to stay on track with the filing of our BLA to the FDA, expected by the end of the year, and our filing in Japan in early 2021. With that plus, our long-term open label extension study has had a high retention rate and continues to provide us the safety data we need for our regulatory findings. Our clinical operations team has been able to open new trial sites as an impressive space during the last few months, including those for our ITP and CIDP trials. We have implemented important measures into these trials to adapt to social distancing requirements and the challenges patients face in visiting sites in person. We quickly enabled our Argenix 117 complement inhibitors to be studied in COVID patients and will continue to be nimble should a second wave of the virus come to Belgium. We are also ready to start our planned phase one trial of Argenix 117 in healthy volunteers any day. Throughout the last six months, we have been able to maintain all lab activities by creating a safe work environment for these essential employees. This has allowed us to keep discovery efforts on track. And finally, the positive phase three ADAPT data readout was a key gating event for us to accelerate our transformation into a commercial organization. This includes the ongoing expansion of our team to prepare for the anticipated US and Japan launches of Avgatigamot. We have been fortunate to make a lot of excellent hires recently. And of course, our increasing commitment to the physician and patient communities has remained a top priority during this time through virtual engagement. Slide five. Before I transition to the rest of our business update, I'd like to say that while COVID-19 has presented a multitude of new challenges over the past few months, we continue to demonstrate our functional agility to adapt as needed. We will not be a company that accepts delays without finding ways to think and work differently and to combat the unforeseen obstacles we are facing. We remain sharply focused on executing our 2021 vision to be a fully integrated immunology company. This is driven by preparing for the successful launch of our first product, executing on our differentiated pipeline of early and late stage development programs, and building out our pipeline through our Immunology Innovation Program, which is on track to yield Algenix 119 later this year. This will be our 10th pipeline asset that is either wholly owned or in the hands of a partner. Let's first talk about Avgar Tigamov, our first-in-class FSHRN antagonist, on slide six. In May, we were thrilled to report positive top-line data from the Phase 3 ADAPT trial, which showed that abgartigamot was well tolerated and able to drive deep responses that support our plan to offer individualized dosing to MG patients. We plan to show the full ADAPT data set later this year at a medical meeting. These meetings are all shifting to virtual and adjusting their programs as necessary, but as soon as we have our confirmed plan, We will share it. To quickly summarize the top line results on slide seven, ADAPT met its primary endpoints with 67.7% of acetylcholine receptor antibody positive GMG patients, which were responders on the MG-ADL score compared to 29.7% on placebo with a p-value of less than .0001. We defined a responder as having at least a two-point improvement on the MG-ARL score for at least four weeks or for five consecutive measurements. This was a pretty high bar for the primary endpoint since we included this durability component. We confirmed this efficacy response on the QMG score as well, with 63.1% of antibody-positive patients responding to F-Gatigamot compared to the 14.1% on placebo on the QMG score with again a p-value of less than 0.0001. To be a responder, patients needed again a three-point improvement for five consecutive measurements. On slide eight, we saw depth of response as measured by minimal symptom expression or reaching an MGADL of zero or one. This is an important measurement for patients and physicians because it means that patients are generally symptom-free. 40% of Edgar-Tigermot-treated antibody-positive patients achieved minimum symptom expression compared to 11.1% treated with placebo. We also show that patients responded quickly and some for an extended period of time. Of the 44 patients who were Edgar-Tigermot responders, 84.1% had a fast response initiated within the first two weeks. Additionally, 88.6% of patients who reached the primary endpoint achieved a response for at least six weeks, 56.8% for at least eight weeks, and 34.1% for at least 12 weeks. On slide nine, Response rates were consistent during a second treatment cycle of Avgatigamov. Remember that 12 patients, or 27%, who responded in the first cycle never required a second cycle. And the start of the second cycle was dependent on the durability of the response in the first cycle. 70.6% of patients who received a second cycle responded, including 36.8% of patients who were not responders in the first cycle. We attribute some of the new responders in the second cycle to the primary endpoint definition. We had several patients that had good responses to abgraticumab in the first cycle but did not meet the high bar of having a clinically meaningful response for five consecutive measurements. We also saw a favorable safety profile that was comparable to placebo and a very high rollover rate from the primary 26 weeks trial to the long-term open-label extension trial called ADEPT+. I would like to contextualize the importance of the ADEPT data readout for our overall FGAT-Tigamot strategy. With these strong data, we are looking to disrupt treatment paradigms in autoimmunity. We know that FCRN is central to IgG regulation, so by targeting it, FGAT-Tigamot could be a logical new therapeutic option for many patients suffering from autoimmune diseases that are driven by pathogenic autoantibodies. In slide 10, we currently have the broadest FCNN pipeline, evaluating four current indications in three distinct therapeutic areas. We are also planning to announce our fifth indication this year. We expect to have the first FCNN antagonist available to MG patients next year, and with this approval we can start a parallel track of investigator-sponsored trials in indications that may not meet the high bar for a registration program but where the biology is solidly understood to be driven by pathogenic IgGs. The ADAPT readout was a gating event to proceed full steam ahead in the preparation for our first commercial launch, the expansion of our commercial team and in our longer-term planning to get FGAT-Tigamot to autoimmune patients as quickly as possible. With that, I would like to shift to our ongoing FGAT-Tigamot trials and those that will start this year. Slide 11. It is a top corporate priority to mitigate disruptions from COVID-19 which are now impacting our ongoing trials. To do this, we have focused on several initiatives for the ADAPT Plus Open Label Extension Trial in MG, and our Advanced and Adhere programs in ITP and CIDP where we have phased enrollment delays. First, we are integrating telemedicine into clinical trial protocols where possible. We are also implementing home infusion opportunities for patients who do not feel comfortable traveling to infusion centers. And finally, We are working to prioritize subcutaneous FGAR-TGMOV. We're using the subcutaneous formulation in the ADHERE-CIDP trial and we'll be implementing SUBQ into ITP as well this year. We'd like to update you on our ongoing clinical trials. In the ADAPT Plus Open Label Extension trial, 133 MG patients remain on study. While we cannot yet quantify the enrollment delays in the ADVANCE or ADHERE programs, we have been pleased with our progress in opening clinical trial sites, virtually, for both. We have opened ADVANCE II for enrollment, but are also in active dialogue with the FDA to assess how best to bring SUBQ to the forefront in the ITP Phase III program. With CIDP, We now expect the go-no-go decision to be a 2021 event. This will happen after the first 30 patients get through part A in the trial and we see if they have a response to F-Gatigamot. Remember that in ADHERE, there is a long screening period to ensure we are getting active CIDP patients. We think this disciplined trial design will pay off in getting the right patients to receive F-Gatigamot and to assess the percent of patients whose disease is antibody mediated. CIDP is also the first trial in which we are evaluating the sub-Q F-Gatigamot that emerged from our collaboration with Helozyme. This collaboration has turned out to be a key strategic decision and competitive advantage. Helozyme's enhanced delivery technology is well validated with a documented safety profile across many biologics. By using this technology, we can achieve at least the same IgG reductions as with the 10 mcg per kick IV formulation, and this in a fast, effortless, single sub-Q injection. We believe this will be an important offering in each of our indications to get to as many patients as possible. On slide 12, for our pemphigus vulgaris program, We will be launching the phase three trial in the second half of 2020. We continue to be very excited about this program after the positive results we showed from the adaptive phase two trial. As a quick reminder of the data, we saw that 90% of patients achieved rapid disease control by a median time of 15 and 22 days for monotherapy and combination therapy. Complete clinical remissions were observed in 70% of patients receiving the optimized dosing regimen determined to be F-Gar-Tigamat dosed at least every two weeks in combination with oral prednisone. 70% of patients receiving 25 mg per kg F-Gar-Tigamat achieved end of consolidation, including patients who prefer to taper their steroid dose rather than potentially achieving a complete clinical remission. Importantly for PV patients, we also saw a tolerability profile that was favorable and consistent with data from previous Avgar-Tigermod studies. Based on these results and on early discussions with physicians, we see how Avgar-Tigermod could fit squarely into current treatment practice to address unmet needs. We will be talking more about the Phase D program as it gets up and running later this year. Moving on to Q-sartuzumab on slide 13. Today, we announced a change to the Q-sartuzumab development strategy, which we believe will best align with a rapidly evolving treatment paradigm in AML. We'd like to first provide a quick reminder of our strategy in partnering this asset. Q-sartuzumab has a unique mechanism of action targeting CD70 and leukemic stem cells. We recognize its potential to be broadly used across AML settings and in MDS patients. Janssen is a global oncology player and an ideal partner to accelerate and expand the global development plan we had agreed on together. Culminate was the first trial launched under the Janssen collaboration, and we had two primary objectives with it. First was dose selection, between 10 and 20 milligram per kilogram Cusartuzumab. We achieved this and have selected 20 milligram per kilogram as the go-forward dose. Second was to design a trial that could be registrational in the case of a stellar response rate, clean safety profile, and durable responses. We knew that Venetoclax data would be a high bar to beat in accelerating our path to registration. We planned so that the second trial launched under the collaboration would be a phase 1b trial of q-sartuzumab in combination with venetoclax and azacytidine. We knew venetoclax had shown early potential in AML and recognized that a combination approach may ultimately be the path forward. Maturing data from Culminate suggests that complete response rates are not likely to exceed those from the VLA-A trial of venetoclax in combination with azacitidine as presented at IHA in June 2020. It is too early to make a judgment on durability of response, and from a tolerability perspective, the profile is consistent with what we have seen in past trials. We currently think the best path forward for quercetuzumab is to study it in combination with Venetoclax to challenge the emerging standard of care. This strategy is supported by positive KOL feedback and by preclinical data that were presented at ASH last year showing synergies between Cusatuzumab and Venetoclax. We hope to amplify the Venetoclax response rates, to extend the duration of the responses, and to provide a tolerable therapy based on early data we have seen. We expect the registration strategy for Cusartuzumab to be determined as we continue to evaluate maturing data across the Cusartuzumab program and EML treatment landscape. The CULMINATE trial alone is unlikely to form the basis of a BLA submission. This means that we will not be enrolling more patients into CULMINATE beyond the 103 already enrolled. We will be prioritizing the Phase 1b trial of the triple combination of CUSA with venetoclax and azacitidine. The trial had been on hold due to COVID-19 but is enrolling again at initial sites as well as new sites. You can see on the slide that the MDS trial remains passed and the ongoing trial in Japan, which was not passed due to COVID, continues to enroll. We cannot provide detailed data today since they are still maturing, but we have committed to show top-line results from Culminate in early 2021, including our rationale for the 20 milligram per kilogram dose selection. We know this is important to you as shareholders, and we are working with Janssen to make this possible. We of course would want the fastest path to registration for CUSA, but we also want to remain disciplined in our development strategies to ensure we are running trials that make sense within the current treatment environment. We believe that by taking a combination approach, we are moving forward in the best possible way to disrupt the AML treatment paradigm. We would now like to shift quickly to our other development programs, including our wholly owned assets, Argenix 117 and Argenix 118, and those fully in the hands of partners. On slide 14, as I mentioned earlier, we are ready to dose the first healthy volunteers in the phase one trial of Argenix 117. This trial was delayed due to COVID, but will now be starting imminently. We believe that by targeting C2, Argenix 117 could be a pipeline in the product against severe autoimmune diseases in the neuromuscular space and possibly in kidney or heme as well. With the phase 1 trial, we will assess PK, PD, free C2 levels for dose selection, bioavailability, and ADA. We will also look at safety and tolerability. Our plan is that once we identified a dose from the phase 1 trial, we can launch parallel trials in autoimmune indications. We have already identified an initial indication, multifocal motor neuropathy, which is a rare typically progressive neuromuscular disease that can greatly impact the quality of patient's life. We also know that complement plays a key role in acute respiratory distress syndrome associated with COVID-19. This has shown to be a deadly complication of the infection. We are working with our Immunology Innovation Program Collaborator Bart Lambrecht to make Argenix 117 available to COVID patients at the Ghent Academic Hospital. This is currently the only site for the trial, and thankfully, there are no longer many available COVID patients in this region. We will continue to move forward with our Argenix 117 development plan in healthy volunteers, and should there be a second wave of COVID in Belgium, we will make the drug available, possibly with more inhuman data at that point. We are also making progress in selecting a lead for ArgenX 118 and are preparing to announce ArgenX 119 this year. On slide 15, as you know, our immunology innovation program has also been a productive engine for assets which we have partnered. ArgenX 112, which is now LP0145 in the hands of Leo Pharma, they had paused a phase one trial in atopic dermatitis due to COVID, but are preparing to reopen sites later this summer. ArgenX114 is now AGMB101 in the hands of Agomap and ArgenX115 is now ABBV151 in the hands of AbbVie. We do not have any update on these molecules today, but you will recall that AbbVie did not pass their trial in solid tumors due to COVID, so this study remains ongoing. We also recently learned from Staten that they initiated dosing in the first in human trial of STT5058 targeting APOC3 for the potential treatment of dyslipidemia. This was formerly Argenix 116. Our commitment as part of our Argenix 21 vision is that we will continue to prioritize our immunology innovation program even as we become a commercial organization. in being a fully integrated immunology company, we want to be as much a commercial organization as an R&D engine. With that overview, I will now turn the call over to Keith for a discussion of our commercial readiness.

Thank you, Tim, and good morning, everyone. Please see slide 16. Today I want to give you an update on some of the ways in which we are preparing for a successful launch of F-Gardigimod in the US in 2021 and in Japan following the US launch. We've been providing you updates on our commercial preparation for over a year now, but following the positive phase three data readout, we have felt a dynamic shift in the work we are doing to get F-Gardigimod to patients as quickly as possible. I can tell you today that all commercial preparation activities are on track across each of the key work streams. As a first step, we are right on track with our BLA filing to the FDA and with our JMAA filing to the PMDA in Japan. The BLA will be filed by the end of 2020 and the JMAA in the first half of 2021. This will be a rolling submission, allowing for us to include longer-term safety data from the ADAPT Plus trial as we have it. We additionally are planning to meet with the FDA in the fourth quarter of this year to talk about our subcutaneous formulation of the Afgardagamod and how we can initiate a bridging strategy to get it into MG patients as soon as possible. This is a top corporate priority. We are right where we need to be in terms of supply chain preparation. We have a long established alliance with Lonza and manufacturing for Afgardagamod is currently out of two different locations One in the UK and one in Singapore. We will be ready with our commercial inventory in time for both our US and our Japan launches. We also have the scale up potential to expand our manufacturing capacity to a third location in the US as we reach even more patients and more geographies. We have entered into collaboration with Cardinal Health as our US third party logistics partner. are in the process of building a strategic and patient-centric network of specialty pharmacy and specialty distribution partners to ensure broad access. Additionally, we're in the early phases of engaging with U.S. payers and will continue these conversations as we progress to launch. Our interactions with physicians continue to be a top priority and we have been able to engage with them virtually over the last several months In sharing our ADAPT data with the physicians, we've heard positive feedback on the potential for an individualized dosing approach since patients with myasthenia gravis have different courses of disease and would benefit from a treatment option that is purpose fit to this variability. On slide 17, we know that in the US, there are 16,000 neurologists who actively treat 65,000 adult MG patients. Of those 65,000 patients, 20,000 with generalized MG. 20,000 of them will likely need treatment beyond steroids and other current options. This is what we see as the target addressable market for F. Gardigamod in the U.S. In Japan, there are about 20,000 total MG patients that suffer from MG and are treated by 200 to 300 neurologists. For physicians, we can't underestimate the importance of education when it comes to launching a first-in-class drug with a new therapeutic modality. We will be educating on the crucial role of the antibody in MG, but also on the central role of FCRN in modulating IgG homeostasis. Our field force will be instrumental in reaching our target physicians. We have already hired an expansive team of medical research liaisons that specialize in the neuromuscular space. This team was crucial during our phase three trial to be a resource for investigators. As we approach launch, they will continue to be a resource for an even broader community of neurologists. We additionally have a growing team of thought leader liaisons who will build relationships with the top MG physicians and support our marketing efforts. We'll start to hire our sales force during the third quarter of this year and expect to have 70 to 80 representatives for our commercial launch. On slide 18, all of our preparatory work to successfully launch F-Gardigimod is ultimately about reaching patients who continue to suffer the effects of MG. We hear from patients about the severe and sometimes life-threatening symptoms of MG. As Argenix continues to grow its presence within the MG community, we want to build awareness of the key unmet needs that still exists. To accomplish this, we have launched a digital disease awareness platform in June called MG United, offering personalized information and resources for those affected by MG. This is the first of many initiatives that will be available to the MG community. We have also been working closely with the Myasthenia Gravis Foundation of America to be available to patients during the COVID-19 pandemic. People living with MG already experience feelings of isolation and the social distancing requirements only amplifies this. Given the number of COVID cases we continue to see, we are planning for a fully virtual launch if necessary. We are grateful that we have had time to acclimate and learn from virtual interactions with each of our key stakeholders and think that this will help us prepare for a successful launch in any setting. With that, I'd like to hand the call over to Eric for a review of our financial results.