Argenx : Half year 2020 financial results and second quarter business update on July 30, 2020

Half-Year 2020

Financial

Results &

Second Quarter

Business

Update

July 30, 2020

Forward-Looking Statements

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This presentation has been prepared by argenx se ("argenx" or the "company") for informational purposes only and not for any other purpose. Nothing contained in this presentation is, or should be construed as, a recommendation, promise or representation by the presenter or the company or any director, employee, agent, or adviser of the company. This presentation does not purport to be all-inclusive or to contain all of the information you may desire. This presentation also contains estimates and other statistical data made by independent parties and by us relating to market size and growth and other data about our industry. This data involves a number of assumptions and limitations, and you are cautioned not to give undue weight to such estimates.

Safe Harbor: Certain statements contained in this presentation, other than present and historical facts and conditions independently verifiable at the date hereof, may constitute forward-looking statements. Examples of such forward-looking statements include those regarding our investigational product candidates and preclinical studies and clinical trials, and the status, plans, timing of expected data readouts and related presentations and related results thereof, including the design of our trials and the availability of data from them, the timing and achievement of our product candidate development activities, our ability to obtain regulatory approval of our product candidates, the expected size of the markets for our product candidates, future results of operations and financial positions, including potential milestones, business strategy, plans and our objectives for future operations. When used in this presentation, the words "anticipate," "believe," "can," "could," "estimate," "expect," "intend," "is designed to," "may," "might," "will," "plan," "potential," "predict," "objective," "should," or the negative of these and similar expressions identify forward-looking statements. Such statements, based as they are on the current analysis and expectations of management, inherently involve numerous risks and uncertainties, known and unknown, many of which are beyond the Company's control. Such risks include, but are not limited to: the impact of general economic conditions, general conditions in the biopharmaceutical industries, changes in the global and regional regulatory environments in the jurisdictions in which the Company does or plans to do business, market volatility, fluctuations in costs and changes to the competitive environment. Consequently, actual future results may differ materially from the anticipated

results expressed in the forward-looking statements. In the case of forward-looking statements regarding investigational product candidates and continuing further development efforts, specific risks which could cause actual results to differ materially from the Company's current analysis and expectations include: failure to demonstrate the safety, tolerability and efficacy of our product candidates; final and quality controlled verification of data and the related analyses; the expense and uncertainty of obtaining regulatory approval, including from the U.S. Food and Drug Administration and European Medicines Agency; the possibility of having to conduct additional clinical trials; our ability to obtain and maintain intellectual property protection for our product candidates; and our reliance on third parties such as our licensors and collaboration partners regarding our suite of technologies and product candidates. Further, even if regulatory approval is obtained, biopharmaceutical products are generally subject to stringent on-going governmental regulation, challenges in gaining market acceptance and competition. These statements are also subject to a number of material risks and uncertainties that are described in the Company's filings with the U.S. Securities and Exchange Commission ("SEC"), including in argenx's most recent annual report on Form 20-F filed with the SEC as well as subsequent filings and reports filed by argenx with the SEC. The reader should not place undue reliance on any forward-looking statements included in this presentation. These statements speak only as of the date made and the Company is under no obligation and disavows any obligation to update or revise such statements as a result of any event, circumstances or otherwise, unless required by applicable legislation.

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Agenda for today

1					Recent Highlights, Including Phase 3 ADAPT Data
2					Efgartigimod: Additional Indications
3					Cusatuzumab and Early Stage Pipeline
4					Launch Preparation
5					Financial Results
6					Q&A

3

Recent Achievements in COVID-19 Environment

• Positive topline readout of Phase 3 ADAPT trial
• Financing to support first commercial launch and advancement of pipeline
• BLA filing remains on track
• Opened new trial sites in ITP and CIDP programs
• Prepared for start of Phase 1 healthy volunteer trial of ARGX-117; enabled complement inhibitor to be studied in COVID patients
• Advanced discovery efforts, including work on ARGX-118 and ARGX-119
• Accelerated transformation into a commercial organization with key hires

Advancing Towards 'argenx 2021' Vision

Commercial franchises

argenx 2021: Reaching patients

Global expansion across three locations

Positive Phase 3 ADAPT data

Late-stage pipeline

FcRn leadership

MG

CIDP

ITP

PV

Cusatuzumab strategic alliance

Immunology breakthroughs	Immunology Innovation Program

Strong balance sheet

Pro-forma cash position of $2.1B

5

Efgartigimod Showed Robust Benefit For Patients With gMG

Primary endpoint met

Meaningful patient

benefit observed

Favorable tolerability

observed

• Statistically significant and clinically meaningful improvement in MG-ADL
• • Fast and deep responses
  • Potential for individualized dosing
  • Safety & tolerability profile comparable to placebo

6

ADAPT Data Show Statistically Significant and Clinically Meaningful Improvement in MG-ADL and QMG

7

ADAPT Data Show Fast, Deep and Durable Responses

Minimal Symptom Expression

Fast Onset of Action

Durable Clinical Benefit

40% of efgartigimod patients achieved minimal

Of the 44 patients who were efgartigimod

Potential for individualized dosing

symptom Expression compared to 11% in

responders, 84.1% had a fast response initiated

Placebo

within the first two weeks

Duration of response

(AChR Ab+ Efgartigimod responders, first cycle)

max response:

25 weeks

12 weeks or more

34.1%

8 weeks or more

56.8%

6 weeks or more

88.6%

4 weeks or more

100.0%

0%

20%

40%

60%

80%

100%

Responder by definition is at least 4 consecutive weeks

8

Repeat Treatment Yielded Equally Strong Benefit

MG-ADL responders

MG-ADL responders

		(AChR-Ab+ patients, first cycle)	27% of			(AChR-Ab+,second cycle)	36.8% of
						P < 0.0001
	80%	P < 0.0001	Efgartigimod patients		80%	Efgartigimod patients
		70.6%
		67.7%		who responded in the				not responding to 1st
	70%		1st treatment cycle		70%			treatment cycle did
responders				and never required a	response				respond in 2nd
60%			2nd treatment cycle	60%			treatment cycle
ADL-	50%				ADL-	50%
40%				40%
MG	30%		29.7%		MG	30%		25.6%
%					%
	20%					20%
	10%					10%
	0%	N=44/65	N=19/64			0%	N=36/51	N=11/43
	Efgartigimod	Placebo			Efgartigimod	Placebo
MG-ADL responder: ≥ 2-point improvement for at least four consecutive weeks during the first cycle					9

	Developing Highly Differentiated Pipeline
							Positive topline ADAPT data
	Program	Target	Indication	Preclinical	Phase 1	Phase 2	Phase 3	Registration	Partner
	IV	FcRn	MG					BLA
Efgartigimod	SC Bridging		MG				Bridging
IV		PV
	IV		ITP
	IV + SC		ITP
	IV		ITP
	SC		CIDP
	TBD		5th indication

Cusatuzumab

+ AZA	CD70	Newly diag. AML (unfit)
		CULMINATE
+ AZA + VEN		Newly diag. AML (unfit) ELEVATE
+ AZA		High-risk MDS
ARGX-117	C2	Autoimmune (MMN)
		COVID-19				Neuro-
						Hem/Onc	Skin
ARGX-118	Galectin 10	Airway Inflammation				muscular
ARGX-119	TBD	TBD

MG: Myasthenia gravis ITP: Immune thrombocytopenia PV: Pemphigus vulgaris CIDP: Chronic inflammatory demyelinating polyneuropathy AML: Acute myeloid leukemia MDS: Myelodysplastic syndromes

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Navigating The Impact Of COVID-19

Telehealth and remote monitoring

Home infusion

SC efgartigimod

133 patients remain on ADAPT+

ADVANCE enrolling up to 156 patients

Decision to expand up to 120-130 patients now in 2021

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PV: Phase 2 Data Support Advancement to Phase 3 Trial

* At least biweekly efgartigimod + corticosteroids @ 0.25-0.5mg/kg	12

Cusatuzumab Strategy

Newly diagnosed elderly patients with AML unfit for intensive chemotherapy

Phase 2 CULMINATE Trial

Cusatuzumab + Azacitidine

Part 1: Dose selection

10 mg/kg

20 mg/kg

• 100 patients enrolled in dose selection
  Trial to stop enrolling new patients

Topline data in early 2021

Phase 1b in Triple Combination

Cusatuzumab

+

azacitidine

+

venetoclax

Development plan strategy to

align with evolving AML

treatment landscape

Trial in Japan

continues to enroll

Higher-risk MDS trial remains on hold

ARGX-117: Sweeping Antibody Targeting C2

Unique Intervention in Complement Cascade

IgG IgM

Mannose sugar

Showcase of Antibody Engineering Capabilities

Sweeping Antibody

Pipeline-in-a-Product

Potential

C1

MASPs MBL

qrs

C4

C2

C3 convertase

C3a C3 C3b

C5 convertase

C5a C5

Option

exercised

C5-C9for C2 (MAC)

Endosome

Lysosome

Cell

MMN

Lupus Nephritis

CAD Classical

GBSCIDP

Ischemia

AMR reperfusion IgAN

MGUS anti-MAG

MN LectinLectin HSCT

C4GN

14

Immunology Innovation Program

Accessing First-in-Class Targets by Collaborating with Leading Research Biologists

argenx	Academic Institutions & Biotechs
Antibody Expertise	Disease Biology Expertise

Co-creating immunology solutions: building beyond each individual contribution

ARGX-112	ARGX-115					ARGX-117	ARGX-118
			Efgartigimod	Novel
Up to	Up to	ARGX-114	ARGX-116	Cusatuzumab	Pipeline-in-
Pipeline-in-	airway
€120M and	$625M and	Profit share	Profit share	50% U.S.	a-product
a-product	inflammation
royalties	royalties				potential
				target
					ARGX-116

ARGX-114

8 assets from Immunology Innovation Program have delivered value to argenx

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Preparing to Bring Efgartigimod to Patients in 2021

Reaching patients, physicians, payors in COVID-19 environment

Supply Chain Readiness

• Global manufacturing scale/flexibility
• Building inventory, 3PL selection
• Specialty pharmacy distribution

The Right Team in Place

• Commercial leads hired
• Field force of MRLs, TLLs, payor teams
• Sales force hires to start in 3Q20

BLA filing by end of 2020 + J-MAA filing in 1H21

Addressable MG Market: Patients Who Need Therapy Beyond Steroids

65,000 adult myasthenia gravis patients in

U.S.

55,000 patients have generalized

form of myasthenia gravis

20,000 patients require

more aggressive

treatment

Estimated Efgartigimod Addressable Market

85%

of people with MG progress* to generalized MG within 18 months

36%

of patients require treatment beyond steroids and ACIs

20,000

*Progression to gMG may be less with early immunosuppressive treatment

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Hope for people living with myasthenia gravis

Eri Abdiel

Railroad inspector and father of six

Living with myasthenia gravis

"When I was younger, they told me I would never walk again … I was able to beat the odds on that, and I intend to beat the

odds on MG."

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Half Year 2020 Financial Results

				Six Months Ended
				June 30,
	(in thousands of € except for shares and EPS)			2020			2019			Variance
	Revenue	€	22,388		€	43,532		€	(21,143)
	Other operating income		8,729			7,767			961
	Total operating income		31,117			51,299			(20,182)
	Research and development expenses		(171,718)			(78,304)		(93,414)
	Selling, general and administrative expenses		(61,644)		(27,462)		(34,181)
	Total operating expenses		(233,362)			(105,767)			(127,595)
	Change in fair value on non-current financial assets		848			-		848
	Operating loss	€	(201,397)		€	(54,467)		€	(146,929)
	Financial income/(expense)		(2,178)		7,210			(9,388)
	Exchange gains/(losses)		199			2,486			(2,287)
	Loss before taxes	€	(203,376)		€	(44,771)		€	(158,605)
	Income tax (expense)/benefit	€	(2,261)	€	(350)	€	(1,911)
	Loss for the year and total comprehensive loss	€	(205,637)		€	(45,121)		€	(160,516)
	Net increase/(decrease) in cash, cash equivalents and current financial assets compared to
	year-end 2019 and 2018		596,977			1,368,229
	Cash, cash equivalents and current financial assets at the end of the period		1,932,798			944,283

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Our Key Priorities

1	File BLA by end of year

2	Commercial preparedness

3	Execute pipeline

4	Expand through Immunology
Innovation Program

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Thank You

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