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ARGX-119

Phase 2

Amyotrophic Lateral Sclerosis | Monoclonal antibody | Neurology |argenx SE|Last Updated: Aug 27, 2026

Target and mechanism

Molecular targetMuSK
Target classProtein
ModalityMonoclonal antibody

Also known as ARGX-119 IV

Success Probability

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Market & Valuation

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Trial Design

RandomizedDouble-BlindPLACEBO_CONTROLLEDDMC
Total Trials1
Total Enrollment60

FDA Designations

No designations recorded

Clinical trial landscape

ARGX-119 · 4 trials · 5 indications

Phase 2 2Phase 1 2
NCT07287982A Study to Assess the Safety, Tolerability, Efficacy, Pharmacokinetics, and Immunogenicity of Intravenous Administration of ARGX-119 in Pediatric Participants Aged 5 to Less Than 18 Years With Spinal Muscular AtrophySpinal Muscular Atrophy (SMA)
RECRUITING60 Analytics
NCT06441682A Safety and Efficacy Study of ARGX-119 in Adult Patients With Amyotrophic Lateral Sclerosis (ALS)Amyotrophic Lateral Sclerosis
ACTIVE NOT_RECRUITING60 Analytics
PHASE2RECRUITING
A Study to Assess the Safety, Tolerability, Efficacy, Pharmacokinetics, and Immunogenicity of Intravenous Administration of ARGX-119 in Pediatric Participants Aged 5 to Less Than 18 Years With Spinal Muscular Atrophy
Spinal Muscular Atrophy (SMA)Unlock trial analytics
PHASE2ACTIVE NOT_RECRUITING
A Safety and Efficacy Study of ARGX-119 in Adult Patients With Amyotrophic Lateral Sclerosis (ALS)
Amyotrophic Lateral SclerosisUnlock trial analytics

Study Endpoints

Primary Endpoints

Incidence of AEs
Up to 124 weeks

Adverse Events

Incidence of SAEs
Up to 124 weeks

Serious Adverse Events

Change in RHS total score from baseline to week 24 of the double blinded treatment period (DBTP)
Up to 24 weeks

The RHS (Revised Hammersmith Scale) is a validated 36-item scale developed to evaluate the spectrum of gross motor function. Maximum total score; 69 (optimal motor function)

Assessment of adverse events (AEs)
Up to week 96
Change from active-treatment baseline over time for 6MWT distance
Up to 72 weeks

The 6-minute walk test (6MWT) measures the distance a participant walks in 6 minutes. Before and after the 6MWT assessment, the participant's blood pressure, heart rate, and SPO2 will be recorded, and the participant's perception of fatigue and dyspnea will be measured.

Number of treatment-emergent adverse events (TEAEs)
up to 157 days for part A, 117 days for part B

Secondary Endpoints

Change from baseline over time for the 6MWT - distance and fatigue index
Up to 124 weeks
ARGX-119 serum concentrations over time
Up to 124 weeks
Incidence of antidrug antibodies (ADA) against ARGX-119
Up to 124 weeks
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Study Design & Arms

AllocationRANDOMIZED
MaskingDOUBLE
ModelPARALLEL
PurposeTREATMENT

Treatment Arms

ArmTypeDescription
DBTP - ARGX-119 IVEXPERIMENTALParticipants receive ARGX-119 IV during the DBTP
DBTP - Placebo IVPLACEBO_COMPARATORParticipants receive placebo IV during the DBTP
ATEP - ARGX-119 IVPLACEBO_COMPARATORParticipants receive ARGX-119 IV during the ATEP. Participants from ARGX-119 IV arm in the DBTP will receive placebo once to maintain the DBTP blinding
ARGX-119 - Dose 1EXPERIMENTALParticipants will receive first dosage level of ARGX-119 intravenously during the double blinded treatment period followed by ARGX-119 in active treatment extension period
ARGX-119 - Dose 2EXPERIMENTALParticipants will receive second dosage level of ARGX-119 intravenously during the double blinded treatment period followed by ARGX-119 in active treatment extension period
ARGX-119 - Dose 3EXPERIMENTALParticipants will receive third dosage level of ARGX-119 intravenously during the double blinded treatment period followed by ARGX-119 in active treatment extension period
PlaceboPLACEBO_COMPARATORParticipants will receive placebo intravenously during the double-blinded treatment period followed by ARGX-119 in active treatment extension period
Double-blinded treatment period - ARGX-119 IVEXPERIMENTALParticipants receive ARGX-119 during the double-blinded treatment period
Double-blinded treatment period - Placebo IVPLACEBO_COMPARATORParticipants receive placebo during the double-blinded treatment period
Active-treatment period - ARGX-119 IVEXPERIMENTALParticipants receive ARGX-119 during the active-treatment period
ARGX-119EXPERIMENTALPatients receiving ARGX-119 IV or SC

Interventions

NameTypeDescription
ARGX-119 IVBIOLOGICALIntravenous infusion of ARGX-119
Placebo IVOTHERIntravenous infusion of placebo
ARGX-119BIOLOGICALIntravenous infusion of ARGX-119
PlaceboOTHERIntravenous infusion of placebo
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Eligibility Criteria

Age Range5 Years to 17 Years
SexALL
Healthy VolunteersNo
Study Sites23

Inclusion Criteria: * Is aged ≥5 to \<18 years when completing the informed consent process, defined as providing informed assent according to local regulations and having a parent or guardian sign the ICF, and can comply with protocol * requirements. * Has documented historical genetic diagnosis o...

Countries:United StatesUnited KingdomBelgiumCanadaDenmarkFranceNetherlandsSwedenItalySpain
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Recent Changes (Last 90 Days)

LOWAug 27, 2026NCT07287982lastUpdatePostDate: changed
LOWAug 27, 2026NCT07287982lastUpdatePostDate: changed
LOWAug 20, 2026NCT07287982lastUpdatePostDate: changed
LOWAug 20, 2026NCT07287982lastUpdatePostDate: changed
LOWAug 20, 2026NCT07287982lastUpdatePostDate: changed
LOWAug 20, 2026NCT07287982lastUpdatePostDate: changed
LOWAug 11, 2026NCT07287982lastUpdatePostDate: changed
LOWAug 11, 2026NCT07287982lastUpdatePostDate: changed
LOWAug 11, 2026NCT07287982lastUpdatePostDate: changed
MEDIUMJul 24, 2026NCT06436742Status: RECRUITING → ACTIVE_NOT_RECRUITING
MEDIUMJul 24, 2026NCT06436742Status: RECRUITING → ACTIVE_NOT_RECRUITING
LOWJul 21, 2026NCT07287982lastUpdatePostDate: changed

Frequently asked questions about ARGX-119

What is ARGX-119 used for?

ARGX-119 is an investigational monoclonal antibody being developed for neurological conditions including Spinal Muscular Atrophy (SMA), Congenital Myasthenic Syndrome, and Amyotrophic Lateral Sclerosis (ALS). It is also studied in healthy volunteers for safety and tolerability. The drug is administered intravenously and is currently in clinical development.

What does ARGX-119 target?

ARGX-119 targets MuSK, a protein involved in neuromuscular signaling. By modulating MuSK activity, the antibody aims to address underlying mechanisms in diseases like SMA and congenital myasthenic syndromes. This target is central to the drug's proposed therapeutic approach in neurology.

Who is developing ARGX-119?

ARGX-119 is being developed by argenx SE, a biopharmaceutical company listed on the stock exchange under the ticker ARGX. The company is conducting clinical trials for the drug across multiple neurological indications, including SMA and ALS.

What phase is ARGX-119 in?

ARGX-119 is in Phase 2 clinical development. It has completed a Phase 1 trial in healthy volunteers and is currently being evaluated in Phase 1b and Phase 2 studies for congenital myasthenic syndrome, ALS, and pediatric SMA. The drug is investigational and not yet approved.

What clinical trials is ARGX-119 in?

ARGX-119 is being studied in several trials: NCT05670704 (completed Phase 1 in healthy volunteers), NCT06436742 (Phase 1b in DOK7-congenital myasthenic syndrome), NCT06441682 (Phase 2 in ALS), and NCT07287982 (Phase 2 in pediatric SMA). These trials assess safety, efficacy, and pharmacokinetics.

Is ARGX-119 the same as ARGX-119 IV?

Yes, ARGX-119 is also known as ARGX-119 IV, referring to its intravenous route of administration. The alternative name is used in clinical trial contexts to specify how the drug is given. Both names refer to the same investigational monoclonal antibody developed by argenx.