argenx SE

switch between treatments. Finally, there is a considerable market opportunity with approximately 16,000 to 17,000 addressable patients in the U.S. alone. We are continuing to enroll patients in the ADVANCE APPEAL, ADHERE, and ADDRESS trials for ITP, CIDP, and PENFIGUS, respectively, which are shown on slide 7, 8, and 9. As we approach enrollment completion, we will be able to provide more granular timing for these results. We recently shared the full results from the Phase II panthicus study in the British Journal of Dermatology. We would encourage you to read this manuscript with data to support our latest understanding of how mCarticumab could fit into the treatment paradigm in panthicus given the observed fast onset of action, favorable tolerability profile and potential to use lower initial doses of corticosteroids and start early steroid tapering. With our newest indications, we are in the final preparation stages to initiate registrational trials. The Bullets-Pentagord trial is on track to start by the end of this year, and myositis is expected to start during the first quarter of 2022, now that we have wrapped up our FDA consultations, and these are depicted on slides 10 and 11. Through our collaboration with Xilab, we are also planning to launch proof-of-concept trials in additional indications. We aren't ready to be public on the plan yet, but in the meantime, XI is preparing to start enrollment of Chinese patients into our ongoing global clinical trials. I'm going to quickly talk about two other important programs in our pipeline, starting with ARGENIX 117, our first-in-class C2 inhibitor. We shared Phase I data from ARGENIX 117 during our R&D day when we explained the reasons why we believe that C2 is the ideal point of intervention within the complement cascade. To name a few, it is upstream of C5, keeps the alternative pathway intact to reduce infection risk, and patients with a genetic deficiency of C2 have a more benign phenotype than that of other complement deficiencies. The Phase I data show the favorable safety profile with both the IV and sub-Q formulations and PKPD profiles that support infrequent dosing. We remain on track to begin the Phase II trial in MMM by the end of this year and look forward to talking about additional indications for this pipeline in a product opportunity next year. Slide 14. We also talked about Argenix 119 for the first time during our R&D day, which is a simple antibody aimed at boosting the neuromuscular junction. This is the latest pipeline product that has emerged from our immunology innovation program. We are excited to share more on this program next year. Before I turn the call to my colleagues, I would like to take a moment to thank our team for their collective efforts as we approach this exciting new stage for Argenix. In particular, I would like to acknowledge Wim Pares. We announced today that Wim will retire after three years with Argenix in March of 2022. In this planned transition, he will become a member of the R&D committee of our board of directors and succession plans are underway for Lutruyen to assume the role in April 2022. We are so grateful to him for his invaluable contributions, the transformation of our R&D organization, and for his humble leadership these past few years. With that, I will turn the call over to Carl to provide a financial update.

Carl? Thanks, Tim. Slide 15 covers our 2021 financial results for the nine-month ended September 30, 2021. I will summarize them here, but they are covered in more detail in today's press release. Total operating income increased to 494.6 million compared to 47.7 million during the same period in 2020. The significant increase was primarily due to the recognition of 315.1 million as a consequence of a termination of a collaboration agreement with Janssen. Additionally, the closing of a strategic collaboration with XyLab resulting in the recognition of $151.9 million in collaboration revenue. R&D expenses increased to $413.3 million compared to $276.4 million in 2020. The increase resulted primarily from higher external research and development expenses. mainly for our FGAR TV mode programs. SG&A expenses totaled $210.2 million for the first nine months for 2021, compared to $113.2 million in 2020. The increase resulted primarily from higher personnel expenses. The change in fair value on non-current financial million so far this year, which is the result of a closing of a Series B financing round of Agomab Therapeutics, in which we have a profit share. Finally, cash, cash equivalents and current financial assets total $2.53 billion as of the end of September, compared to $2 billion on December 31, 2020. I now like to turn the call over to Keith Woods to provide an update on our commercial launch preparation. Keith?

Thanks, Carl. Flight 16, please. We are on track with our preparations for the global launch of F-Guard Tigamod in MG. Based on our December 17th PDUFA date in the U.S., we anticipate an effective launch date in January 2022. In Japan, we anticipate an approval in the first quarter of 2022, followed by a commercial launch three months later once we have price set. In Europe, we expect to have an approval in the second half of 2022, and then we will negotiate price and reimbursement on a country-by-country basis, and this is a process that can take anywhere from 12 to 36 months. Slide 17, please. We are also excited to announce that as of today, our U.S. and Japan field teams are fully on board. We are currently conducting training and account profiling to ensure that we are prepared to reach patients, providers, payers, and other stakeholders upon approval. We have 70 territory business managers in the US and 24 in Japan, but our full field force in the US will be a team of 146 people. In addition to the territory business managers, the team also includes the regional business directors, nurse case managers, case coordinators, medical research liaisons, thought leader liaisons, field reimbursement managers, regional account managers, and national account directors. We continue to be impressed with the level of talent, experience, and enthusiasm the new team members bring, and we see their shared commitment towards the patient. We know that the investments we are making in our team and our other infrastructure now will benefit us for the long term because we will recognize economies of scale as we expand to each commercial franchise across new indications and with new assets. While we feel we have assembled a best in class team that is equipped to handle the challenges associated with this potential launch, we know that there are certain aspects that are outside of our control. Namely, the COVID pandemic continues to create uncertainty for our teams and the communities we hope to serve. We are building a launch plan that incorporates both virtual and in-person components based on feedback from physicians on how to optimize our interactions with a hybrid approach. We also know that it may still be difficult for patients to easily access the facility for treatment. So in addition to building a network of infusion centers, we have also built a home infusion network. In addition to the unique COVID scenarios we are facing, there are additional challenges associated with a first launch of a product. As we've said many times, we will not have a J code at launch. We will apply for one shortly after approval in the first quarter and expect to have a dedicated J-code in place by quarter three of 2022. This may slow things down in the first two quarters with prescribers having to go through a reimbursement appeal process. Second, F-gartigamide will be a first-in-class therapy. Targeting FCRN is a novel mechanism of action that many physicians are not familiar with and do not have hands-on experience. It will take time to get to the physicians, patients, and payers and educate until they are comfortable with this new, innovative class of medicines. Finally, we are limited in the extent of engagement we can have with stakeholders before an actual approval. For these reasons, we continue to believe that we are positioned for a launch with a gradual, steady growth, and it will take time to reach the full potential of EFGAR-Tigamot and MG. With all this in mind, I want to make it clear that our overall outlook on the potential for FGAR-Tigamot and GMG remains unchanged. We know that this community is in need of alternative options and that we have a compelling value proposition based on the strong ADAPT data, the potential for individualized dosing, having both IV and sub-Q formulations, and our growing safety database. Slide 18, please. In summary, We are excited and prepared for the global launch of FGAR-Tigamod and see the significant unmet need that people living with MG still face. We learned from our real-world evidence study that people living with this disease are negatively impacted on multiple levels, physically, mentally, socially, and emotionally. In fact, 92% of survey responders agree that there is a significant need for new treatments. and 96% of responders were hopeful for options with fewer side effects. Participants in the study were most likely to experience problems with double vision, breathing, and eyelid droop severity. We also learned that 42.4% of participants had depression score high enough to meet the threshold for this diagnosis, as well as 52.4% for anxiety. In a separate Argenic-sponsored patient burden survey, 51% of patients stopped working entirely due to their disease. We have been fortunate enough to spend considerable time with the MG community over the last several years. Hearing about these challenges, We are hopeful that F-gartigamide can be a new treatment option for people living with GMG. I'll conclude there and turn the call back over to Tim for final remarks. Tim?