Argenx Se · EURONEXT:ARGX
Argenx — Investor Day (July 2024)
156 slides from Argenx's investor day presentation, Jul 16, 2024
Slide 1: argenx
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Slide 2: Welcome & Opening Remarks
§ Welcome & Opening Remarks Beth DelGiacco /// Vice Presider e Communications & Investor Relations argenx
Slide 3: Forward Looking Statements
Forward Looking Statements “Tia presentation has been prepared by argent se Corgan” of he “compény) for ntomationel purposes only and na fren other purpose. Noting coriained inthis presentation iso sh cr 7 Cetin statements contained in tis presentation, cher han present and historical fects and cond
Slide 4: argenx *
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Slide 5: Agenda
Welcome and Opening Remarks Beth DelGiacco argenx Vision 2030 ~~~ Tim Van Hauwermeiren Immunology Innovation Peter Ulrichts, Karen Silence Clinical Development ==" Lue Truyen Myositis ~~ Leentje De Ceunind Sjogren's Disease v= ile Jacobs ‘Sjégren’s Disease KOL Panel ~~~ Julie Jacobs, Prof. Simon Bow
Slide 6: argenx Leadership Here Today
argenx Leadership Here Today Peter Ulricht® PH.D) (Chet Scent Ofiee @\- Inge Van de Walle Ph.D. ReveacnFetor argenx S
Slide 7: Thought Leaders Here Today
Thought Leaders Here Today Simon Bowman, Ph.D., M.B.B.S., F.R.C.P. Patrick Kwon, M.D Institute of Inflammation and Ageing, Clinical Associate Professor, Neurology, 2 University of Birmingham New York University Grossman School of Medicine argenx
Slide 8: Key Themes for Today
Sy Key Themes For Today argenx™ Our innovation model Leadership in FcRn Expansion of our immunology pipeline Setting a new standard in MG and CIDP Next wave of efgartigimod indications Building weight behind empasiprubart Vision 2030 - path to 50,000 patients
Slide 9: Vision 2030
t Vision 2030 E m Van Hauwermeiren /// C itive Officer argenx
Slide 10: Vision 2030
Vision 2030 New Molecules Labeled Patients on in Phase 3 Indications Treatment COMMITMENT TO OUR TRANSFORMATION MISSION (coninous Pipeline of imovaon ) < Leadership in FcRn ) C Disciplined Scaling +) argenxY 10
Slide 11: Entrepreneurial spirit — calculated risk based on data
o Entrepreneurial spirit — calculated risk based on data Est. 2008 74 | I i ‘ion thi Ih AFRCNX & OO nvscteresrreation (@) Execution excellence argenxY
Slide 12: Our Understanding of Human Immunology is Growing Exponentially
Our Understanding of Human Immunology is Growing Exponentially
Slide 13: Our Innovation Playbook
Novel Disease Biology Insights Foundational Immune Targets, Our Innovation Playbook Best-in-Field Antibody Engineering First-in-Class Antibodies Pipeline-in- a-Product Development Differentiated Patient Outcomes,
Slide 14: Co-Creation is Our Innovation Formula
Co-Creation is Our Innovation Formula WORLD CLASS ANTIBODY ENGINEERING CAPABILITY DISEASE BIOLOGY INSIGHTS \V-REGION CAPABILITIES ‘SIMPLE ‘Antibody™ Platform
Slide 15: Our Innovation Model Has a Strong Track Record
Our.Innovation Model Has A S Track Record 8/12 demonstrated IL6 cD70 human POC.” * 9 first-in-class targets 5 partnered Sd Broad applicability across 35+ indications argenx*
Slide 16: Innovation Through Co-Creation Exists Across argenx
Innovation Through Co-Creation Exists Across argenx ltl elektrofi it SIOVIA zai-=>- Pi mo nifed (Ceees.ciop ! panies Decors — Lone BEBE _Z/92 IFiccxyne re odebt adheres alkivia My GAR atk 16
Slide 17: Successful Execution of Our Vision 2025
Successful Execution Of Our Vision 2025 Growing autoimmune market Efgartigimod available globally Vibrant franchises Efgartigimod in development in 15 indications ARGX-117 in late-stage trials Proof-of-concept in ARGX-119 New asset each year from IIP Rooted in Science through our IP Committed to our
Slide 18: VYVGART is a Global Blockbuster
VYVGART is a Global Blockbuster VYVGART generated >$1B in second year of launch Approved in 3 indications globally Leading market share among MG branded biologics On launch trajectory to leading autoimmune franchise yuveart Dupxent >> Rituxan Rituxima &s argenx 8
Slide 19: Robust Pipeline of Multi-Indication Assets
Robust Pipeline of Multi-Iindication Assets Efgartigimod in 15 indications Empasiprubart in 4 indications ARGX-119 in CMS and ALS 4 new INDs by end of 2025 Proram viveart VVCART Hytrulo rc htm Dera Hermano C0 somo ae ‘i nr i etn 88.06) uo ere ‘nye fron typ Prase —__ProofofConcept Registrations! Comm
Slide 20: Reaching Patients Globally with VYVGART Franchise
Reaching Patients Globally with VYVGART Franchise >10,000 patients on treatment* VYVGART and VYVGART Hytrulo? approved across 3 continents within one calendar year Reaching Patients Across the Globe ») vevcart ©) VWVGART'Hytrulo
Slide 21: Staying True to our Scientific Roots
Staying True to our Scientific Roots From lIP to marketplace, science is our common language Robust patent portfolio Advanced our scientific expertise with peer reviewed publications in top medical journals nature THE LANCET | Neurology Neurology’ ® trontiers in Immunology argenxY 2
Slide 22: Creating Superior Shareholder Value on our Path to Self-Sustainability
Creating Superior Shareholder Value on our Path to Self-Sustainability Rapid transition to sustainable company Disciplined scaling argenx™ Total Shareholder Return since IPO in 2014 27.4 $B 5.6 Gross proceeds Market cap from equity raises argenx TSR ~5,000% 2
Slide 23: Vision 2030
Vision 2030 New Molecules Labeled Patients on in Phase 3 Indications Treatment COMMITMENT TO OUR TRANSFORMATION MISSION (coninous Pipeline of imovaon ) < Leadership in FcRn ) C Disciplined Scaling +) argenxY 2
Slide 24: Our Horizons
t Our Horizons } revs @ (Anti-FeRn) ARGX-109 ARGK.121 Additional Now Molecule Molecules in Phase 3 Que on EARLY PIPELINE LATE PIPELINE
Slide 25: Labeled Indications In-Market as Next Wave of Potential
Labeled Indications In-Market as Next Wave of Potential auictiee © Curenty approved indications © VYVGART © Empasiprubart © ARGX-119 a argenx
Slide 26: Innovation
Innovation meaning unle reaches patients and provides real: benefit argenx *
Slide 27: Pipeline horizons
ARGX-213 (Ant-FeRa) ARGX-109 mwoskagont (Ant-L-8) \ ARGX-121 ) CAnti-iga) O24 ene EARLY PIPELINE LATE PIPELINE
Slide 28: argenx *
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Slide 29: Blueprint for Innovation
Blueprint for Innovation Next Wave of First-in-Class Immunology Targets argenx
Slide 30: ARGX-113, ARGX-117, ARGX-119
ARGX-113. ARGX-117 ARGX-119 Efgartigimod Empasiprubart
Slide 31: ARGX-117
ARGX-117
Slide 32: Unraveling Central Role of C2 in Complement Cascade
Unraveling Central Role of C2 in Complement Cascade Novel Di etsideer Biology Insights ad Engineering 2
Slide 33: POC ARDA Data
POC ARDA Data 2
Slide 34: Unique complement toolkit
Unique complement toolkit C2-KO and human C2-transgenic mice >30 complement assays in house across different species Various species cross-reactive anti- C2 mAbs for translational models Gytiser “Sirai* Extensive network of experts Advancing science
Slide 35: Empasiprubart in Action
_Empasiprubart ~ in Action argenx
Slide 36: C2 is Uniquely Positioned in Complement Cascade
C2is Uniquely Empasiprubart Positioned in Complement Cascade 8 argenxY
Slide 37: Empasiprubart Demonstrates Long Half-life and Sustained Pharmacodynamic Effect
Empasiprubart Demonstrates Long Halflife and FEeawatier la, Sustained days as of 30 mg/kg dose Pharmacodynamic effect Complement C2, Fee (mat) oot Free C2 levels visas TSS % Fy “Time ator rtinfsion (wooks) = Preto “ Erpantrba 30 mag © Empasinbart 10 mat = Enpasprutar 60 moka a7
Slide 38: Immunology Innovation Program: Model of Co-Creation
Immunology Innovation Program: Model of Co-Creation Best-in-Field Antibody Engineering First-in-Class Antibodies ~ Pipeline-in- a-Product Development Differentiated Patient ~ 4 28
Slide 39: ARGX-113, ARGX-117, ARGX-119
ARGX-113. ARGX-117 ARGX-119 Efgartigimod Empasiprubart
Slide 40: ARGX-119
ARGX-119
Slide 41: Strengthening the Neuromuscular Junction through MuSK Activation
Strengthening the Neuromuscular Junction through MuSK Activation m Best-in-Field Pipeline-in- Novel Disease ‘Antibody a-Product Biology Insights Engineering Development
Slide 42: First-in-Class ARGX-119
Firstin-Class i ARGX-119 Differentiated Patient \A Phase 1 Data Support POC Studies 2
Slide 43: Unique MuSK toolkit
Unique MuSK toolkit MuSK phosphorylation assays ACHR clustering assays Various binding assays Various in vivo neuromuscular disease models including DOK7 CMS mice Human in vitro ALS NMJ co-cultures. 7 Unnerate eth stoned ABO wisn. TRAYA ED argenxY Extensive network of experts 4a
Slide 44: ARGX-119 Boosts Functioning of NMJs by Improving AChR Clustering
ARGX-119 Boosts Functioning of NMJs by Improving AChR Clustering AGRIN MuSK phosphorylates downstream signaling components MuSK-induced clustering of ACHRs and muscle contraction upon Acetyicholine binding Retrograde/signal to stimulate presynaptic differentiation
Slide 45: CMS Rationale
CMS Rationale Early Neonatal Lethality and Disease Relapse are Rescued by ARGX- 119 in DOK7 CMS mice Diminished MuSK phosphorylation in DOK7 CMS Leads to lethal weakness of diaphragm muscles MuSK activation by ARGX-119 rescues phenotype argenx®
Slide 46: ALS Rationale
ALS Rationale Activation of MuSK Signaling Slows Muscle Denervation and Improves Motor Function Slowdown of muscle denervation Improving motor system output argenxY 46
Slide 47: Path Forward for ARGX-119
CMS Phase 1b Phase 2a reALiSe to start in 4Q24 to start in 4Q24 Proof of Biology Intra-Patient Dosing NATURAL HISTORY STUDY ONGOING Path Forward * for ARGX-119 Innovation Within Discovery MSean (MScan-<derived Motor Unit Number) argenx
Slide 48: Immunology Innovation Program: Model of Co-Creation
Immunology Innovation Program: Model of Co-Creation Best-in-Field Antibody Engineering First-in-Class Antibodies ~ Pipeline-in- a-Product Development Differentiated Patient ~ 4 46
Slide 49: ARGX-113, ARGX-117, ARGX-119
ARGX-113. ARGX-117 ARGX-119 Efgartigimod Empasiprubart
Slide 50: ARGX-113
ARGX-113
Slide 51: Leadership in FcRn
Leadership in FcRn Best-in-Field Antibody Pipeline-in- Novel Disease ercdict Biology Insights Engineering Development * as of Q1 2024
Slide 52: Best-in-Field Antibody Engineering
Best-in-Field Antibody Engineering Pipeline-in- a-Product Development Novel Disease Biology Insights Foundational Firstin-Class Differentiated Patient Immune Targets ‘Antibodies Outcomes FeRn Efgartigimod >10,000* patients on VYVGART * as of Q1 2024
Slide 53: Unique FcRn toolkit
Unique FcRn toolkit Differentiating binding assays Predictive cellular assays Extensive network of expe! University of Bsoiitharnpton S$ Sanquin Authera Expert structural biology Innovative hFcRn/hAlbumin transgenic mouse models Advancing Science
Slide 54: Endogenous Efgartigimod
Endogenous Efgartigimod ee IgG interaction Efgartigimod interaction Efgartigimod Binds to FcRn in prea, JOR ee Same Formation | ia eines: vi As Endogenous a NM HR IgG 0 Call membrane argenxY
Slide 55: Efgartigimod is Unique Among FcRn Antagonists in How it Binds
Full-length monoclonal Endogenous IgG Efgartigimod anti-FcRn antibodies Efgartigimod is Unique Among | FeRn Antagonists =n tf ste in How it Binds Call membrane argenxY
Slide 56: Unique Binding of Efgartigimod Leads to Differentiated Intracellular FcRn Trafficking
Unique Binding of Efgartigimod Leads to Differentiated Intracellular FeRn Trafficking 0 3 6 9 12 15 18 21 24 Incubation time (h) No interference of efgartigimod with albumin binding and recycling No degradation of FeRn induced by efgartigimod y argenx=
Slide 57: Unique Binding of Efgartigimod Positively Impacts Albumin Levels and Safety Profile
Unique Binding of Efgartigimod Positively Impacts in vivo Albumin Levels and Safety Profile Efgartigimod does not lead to albumin reductions in vivo HSA (% of baseline) 18 10 5 0 5 10 18 2 2 30 35 Days postinjection 3 Efgartigimod treatment results in a favorable safety profile in the clinic No albu
Slide 58: Evolution of a Novel Target to a Novel Platform
: . : . : ehh Pipeline Next Antagonist in ‘Sweeping in-a-Product FcRn Portfolio Antibody Platform \ Je JL Je J Evolution of a Novel Target to a Novel Platform Novel Target argenxY ss
Slide 59: Next Wave of First-in-class Immunology Targets
Next Wave of First-in-class Immunology Targets //, Preclinical uct Development Karen Silence argenx
Slide 60: Leverage Albumin to Broaden FcRn Targeting Portfolio
Leverage Albumin to Broaden FcRn Targeting Portfolio Deep Knowledge of FcRn Biology reasoeo Builds New Pipeline Candidates ‘oatumin VHH ARGX-213 argenxY
Slide 61: Improving Pharmacokinetics of Efgartigimod Through Binding to Serum Albumin
Improving Pharmacokinetics of Efgartigimod Through Binding to Serum Albumin ‘Albumin and lgG recycle via different binding sites © repizrones, 6x 219 reves ws er binding to Fen © 1:6 cearaded in ysosomes eto © 170x218 reyes vin Fen aun albumin VEE argenxY ™
Slide 62: ARGX-213 is Designed For Optimal FcRn Binding
ARGX-213 is Designed For Optimal FcRn Binding and Equipped with Unique Features Prototype ARGX-213 Two-armed ARGX-213 . Invention \ ‘i Valency VHH_ Aom® Position VHH Linker length Cell membrane @ Enhanced Pk @ Enhanced Pk @ Enhanced Pk Sustained PD @ sustained PD @ sustained PD Albumin sparing Album
Slide 63: ARGX-213 Can Achieve Extended Dosing
— tied. 0 9 wey 6 noe 12s my 820,24 mg, oly ARGX-213 has increased half- «+ \\ 8902.48 ga monty life compared to efgartigimod ARGX-213 Can \ resulting in prolonged PD effect Achieve Extended ° : fico Dosing ® eG) Simulations predict potential for monthly dosing 0 12 2% 36 48 6 72 Bf Time (day) argen
Slide 64: Path Forward for ARGX-213
Path Forward Finalize GLP Beemipeloical Phase ‘ to Start for ARGX-213 \ Tox Study Trial ae in 2H25 argenx *
Slide 65: Deep Knowledge of FcRn Biology Builds New Pipeline Candidates
Deep Knowledge of FcRn Biology Builds New Pipeline Candidates Leveraging Efgartigimod Backbone to Build New Class of Highly Potent Sweeping Molecules gAtargetng soocTargeting 2006-Targeting Fab Fab Fab §.- FoABDEG™ reaapec™ ARGX-121 ARGX-XXX ARGX-XXX argenxY
Slide 66: ARGX-121 Mode of Action
ARGX-121 Mode of Actions o% |. FeRn-mediated IgA degradation Il Blocking of IgA:CD89 mediated signalling cag RE @ ‘ARGX-121 binds to IgA (1-3 mg/m!) *+ Monomeric IgA binds with low affinity to CD89 (Fea!) but upon formation of immune complexes it binds with © Enhanced endocytosis of ARGX-121 IgA com
Slide 67: ARGX-121 Innovative Design Breakthrough
ARGX-121 Innovative Design Breakthrough Prototype ARGX-121 Two-armed ARGX-121 One-armed IgGt-LALAPG-ABDEG™ Invention x Invention Fern Affinity at pH 7.4 and pH 6.0 Immune complex formation 3 FeRn degradation FcRn occupancy eopeeneeth —— i —— i Rex making mane compos isk makirg mune compere Fe deacon
Slide 68: ARGX-121 Rapidly and Drastically Impacts Circulating IgA Levels
ARGX-121 Rapidly and Drastically Impacts Circulating IgA Levels IgA (% of baseline) 0 4 2 2 56 7 Time after 1st dose (day) >90% IgA reduction within 1 week Enables flexible dosing Broad therapeutic potential
Slide 69: IgA is Fundamental in Many Diseases & IgA nephropathy
IgA is Fundamental in Many Diseases & IgA nephropathy ARGX-121 oe ic Pipeline-in-a- SA cela disease Product Potential cD89 » Ngee: Rheumatoid arthritis ‘Systemic lupus erythematosus t) [SR Unear pA buns disase Mucous membrane pemphige Pe Deratts herpetormis. Bullous pemphigoit argenxY
Slide 70: Path Forward for ARGX-121
Path Forward FinalizeGLP | “aha cal Phase 1 to start for ARGX-12 Weeox study, 4H25 in 2H25 argenx *
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Slide 72: Clinical Development
§ Clinical Development argenx
Slide 73: Clinical Development: Bridging Innovation and Unmet Patient Need
Clinical Development: Bridging Innovation and Unmet Patient Need @ ©
Slide 74: Rapidly Scaling our Clinical Footprint
asfectonPHASE) == 1 = 2—= 3m ams | Rapidly Scaling our Clinical Footprint argenxY 2016 2017 2018 2019 2020 2021 2022 2023 2024
Slide 75: Pioneering in MG to Set New Standard for Treatment
Pioneering in MG to Set New Standard for Treatment Earlier treatment lines : Seronegative, Ocular MSE >50% Reduction oss studies steroid ust Highest MG-ADL. ‘Sustained responses Broadest safety and QMG response across dosing schedules database (rt adr song eed oice LC | Geil lee Neurology’ THE LANCE
Slide 76: Applying Our Innovation Approach to Clinical Development
Applying Our Innovation Approach to Clinical Development Innovation Co-Creation | Execution Evidence = Patient Insights Speed Generation A) Build broadest data to guide treatment Patient engagement in trial decisions for patients design and execution Bring medicines to patients as quickly as possibl
Slide 77: Ocular and Seronegative MG
Ocular and Seronegative MG Evidence Patient Generation Insights ADAPT/ADAPT+ Significant need Efficient studies Real-world data Lack of innovation Significant - = underserved population argenxY
Slide 78: Sjögren's Disease
Sjogren’s Disease p rhostupy argenxY Evidence Generation Depth of data from RHO study Leveraging all FcRn data Patient Insights Endpoint selection PRO measures Phase 3 to start by end of 2024
Slide 79: Immune Mediated Myopathies (IMM)
Immune Mediated Myopathies (IMM) argenxY Evidence Generation ‘Subtype selection based on pathogenic IgG rationale Patient Insights Common TIS endpoint Seamless Phase 2/3 Study with interim analysis
Slide 80: Multifocal Motor Neuropathy (MMN)
Multifocal Motor Neuropathy (MMN) node Z mat LP iMMersioN Multifocal Motor Neuropathy argenxY Evidence Generation Robust PoC from ARDA EoP2: endpoint alignment Patient Insights Natural history study exceeds 100 patients to date Leveraging Ph2 and iMMersioN to accelerate recruitment
Slide 81: CIDP is 4th Indication for Empasiprubart
Slide 82: Bringing Innovation to Patients
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Slide 84: Efgartigimod in Myositis
Slide 85: Melissa Living with Myositis
Slide 86: Idiopathic Inflammatory Myopathies (IIM) or Myositis
Slide 87: Myositis Specific Autoantibodies Associated with Different Clinical Symptoms
Slide 88: Myositis Auto-antibodies are Pathogenic
Slide 89: IMNM Antibodies Trigger Muscle Damage and Impair Muscle Regeneration
Slide 90: Efgartigimod Reduces IMNM Antibodies and Restores Mouse Muscle Function
Slide 91: Efgartigimod Prevents Necrosis & Allows Regeneration of Muscle Fibers
Slide 92: Efgartigimod Leads to Full Regain of Muscle Function in the IMNM Mouse Model
Slide 93: Phase 2 / Phase 3 Adaptive Basket Trials with Efgartigimod in IMNM, ASyS, DM
Slide 94: Path Forward for Myositis
Slide 95: Slide 95
Slide 96: Efgartigimod in Sjögren's Disease
Slide 97: Lisa Living with Sjögren's Disease
Slide 98: Sjögren's Disease
Slide 99: Systemic Manifestations of Sjögren's Disease
Slide 100: Auto-antibodies are Key Players in Sjögren's Disease
Slide 101: RHO Trial: Proof-of-Concept in Sjögren's Disease
Slide 102: Primary Endpoint: CRESS
Slide 103: Efgartigimod Demonstrated Effect on Primary Endpoint CRESS
Slide 104: Observed Treatment Effect in 4 Items of CRESS
Slide 105: Secondary Endpoint: STAR
Slide 106: Efgartigimod Demonstrated Effect on STAR
Slide 107: Secondary Endpoint: ESSDAI
Slide 108: Efgartigimod Demonstrated Effect on ESSDAI
Slide 109: Efgartigimod Shows Potential to Break Loop of Immune Activation and Tissue Damage
Slide 110: Patient Narrative Confirms Effect of FcRn Inhibition with Efgartigimod
Slide 111: Proof-of-Concept Established in Sjögren's Disease
Slide 112: Consistency of Data Demonstrates Path Forward
Slide 113: Path Forward for Sjögren's Disease
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Slide 115: Sjögren's Disease
Slide 116: Sjögren's Disease
Slide 117: Q&A
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Slide 119: Empasiprubart
Slide 120: Brenda Living with MMN
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