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argenx SE Investor Day Presentation Deck

IMNM Autoantibodies Trigger Muscle Damage and Impair Muscle Regeneration Classical complement activation →>> Myolysis (muscle enzymes e.g., CK) argenx 2 Anti-SRP/-HMGCR autoantibodies bind 1 muscle fiber Complement dependent Necrosis 3 Myofibre Allenbach, Nature Reviews Rheumathology. 2020 Macrophage recruitment and activation Membrane attack complex -C1q Complement activation Necrosis Induction by proinflammatory cytokines Atrophy IL-1 IL-6 . TNF Y Autoantibodies Myoblasts Muscle damage Impaired muscle regeneration 4 Low IL-4 IL-13 Macrophage recruitment and activation IL-1, IL-6, TNF atrophy Regeneration Myotube SRP antigen - HMGCR antigen 00 00 00 00 Young myofibre 5 Blocking myotube formation Complement independent 26

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