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argenx SE Investor Day Presentation Deck

● ● Autoantibody Levels Correlate to Indicators of Myositis: CK Levels and Muscle Strength Patients were treated with standard of care immunosuppressants CK (U/L) 8000 argenx 6000 4000 2000 7500 5000 2500 Patient 1 CK (U/L) -Anti-SRP54 level (AUJmL) 600 Patient 5 800 1600 1200 800 -400 1000 8000 4000 2000 +0 200 400 600 800 1000 1200 1400 Days 1500- 12000 100001 1000- 500- 7500- 5000 2500- 0 200 400 100 200 Patient 2 12000 Patient 6 9000 6000 3000 +0 600 800 1000 1200 400 300 200 100 0 300 400 500 600 Days 1200- 600- 300 10000 7500 5000 2500 0 200 Patient 3 400 600 Patient 7 200 Days 4000 3000 2000 1000 +0 800 1000 1200 12000 9000 6000 3000 40 600 4000 3000 2000 1000 8000 6000- 4000- 2000- 50 Patient 4 100 Patient 8 200 Days 150 400 Benveniste et al., Arthritis Rheumatol. 2011; Stone et al., Arthritis and Rheumatism 2007; Allenbach et al, Medicine (Baltimore). 2014; Muro et al., Rheumatology 2012 4000 3000 2000 1000 200 75 50 25 to 600 Similarly, anti-HMGCR, -Jo-1 and -Mi-2 titers correlate with CK levels and muscle strength Anti-MDA5, -Jo-1 and -Mi-2 titers correlate with extramuscular disease activities (e.g. interstitial lung disease, skin disease) Anti-SRP54 level (A.U./mL) 31

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