Therapeutic goal

achieve the best possible disease control while restoring or maintaining the patient's quality of life1

  • 1

    DEFINE

    level of disease control, based on severity of clinical symptoms

    ocular

  • 2

    RECOGNIZE

    (highly) active gMG (including refractory MG)

    ocular

  • 3

    EVALUATE

    disease activity based on clinical symptoms, fluctuations, exacerbations or myasthenia crises, duration, and remissions

    ocular

  • 4

    BASE treatment decisions on disease activity

    taking into account MGFA status, age, thymic pathology, antibody status, and response to symptomatic and disease-modifying therapies

    • OFFER patients disease-modifying (immuno)therapy as well as symptomatic therapy
    • START with lower-potency medications with long-established and relatively safe risk profiles in patients with mild or moderate MG, then escalate to more potent medications if needed
    • USE higher-efficacy medication for highly active disease, early after diagnosis if needed
    ocular

  • 5

    AIM

    for best possible disease control and quality of life

    ocular

Definition of MG disease 1

(Highly) active (including refractory MG) despite adequate course-modifying and symptomatic therapy is defined as any of the following

1
MGFA status ≥IIb and/or ≥ 2 recurrent severe exacerbations or myasthenic crises in the year since diagnosis
2
MGFA status ≥IIa and severe exacerbation or myasthenic crisis in the last year
3
MGFA status ≥IIa for ≥2 years

Levels of MG disease control1

1234
Full disease control
No disease activity, no residual symptoms
Full disease control with no detectable disease activity
Minimal residual symptoms with stability, incomplete remission
Incomplete disease control with disease activity
Instability, deterioration, fluctuation with residual symptoms, continuous new or developing symptoms, +/- fluctuations. +/- crises
No disease control with high disease activity, including refractory MG
Continuous symptoms with or without crises or deteriorations, resistance to therapy

MG therapy should be based on current MGFA status, age, thymic pathology, antibody status, and disease activity1

Disease Assessment
  • Based on disease severity and activity
  • Includes current MGFA status
  • Distinction between mild/moderate versus highly active disease
  • Medical assessments (e.g. QMG score, MG-ADL, MG-QOL15r)
  • Should be performed regularly
Determination of Disease Activity
  • Severity of clinical symptoms
  • Duration of symptoms
  • Tendency to remit
  • Residual symptoms
  • Presence or number of crisis-like exacerbations/crises
  • Fluctuations in clinical presentation or residual signs of disease
Antibody Status
  • AChR+, MuSK+, LRP4+ or seronegative MG
treatment

Treatment goals focus on disease and symptomatic control and minimising AEs1

  • Continuous control of disease activity and symptoms (MGFA-PIS of Minimal Manifestations or better)
  • Minimise adverse events (no higher than Grade 1 AEs)

Introducing our doctors

Dr. Alanood Alsolaihim
Dr. Alanood Alsolaihim
Consultant, Neuromuscular Medicine & Electromyography Section Head, Neuromuscular section, neuroscience center of excellence.Medical Quality, Director of Neuroscience Center.
Dr. Raed Alroughani
Dr. Raed Alroughani
Consultant Neurologist, Director of MS Clinic Amiri and Ibn Sina Hospital Kuwait City, Kuwait

Direct me to the GCC Guidelines

Go Now

MG, myasthenia gravis, gMG, generalized myasthenia gravis; MGFA, Myasthenia Gravis Foundation of America, AChR+, anti-acetylcholine receptor autoantibody-positive; LRP4+, anti-low-density lipoprotein receptor-related protein 4 autoantibody-positive; MG-ADL, Myasthenia Gravis-Activities of Daily Living; MG-QOL15r, Myasthenia Gravis Quality of Life 15 Items Scale; MuSK+, anti-muscle-specific tyrosine kinase autoantibody-positive; QMG, Quantitative Myasthenia Gravis

References:

1. Wiendl H et al.. Guidelines for the management of myasthenic syndromes. Therap Adv Neurol Disord 2023 .