Therapy goals
- Stabilization of quality of life
- Improvement and maintenance of independence in terms of mobility, participation in social life and independent care.
Therapy recommendations
- To reduce the autoimmune response – immunosuppression must be maintained for several years, often for life
- 1st-line agents: immunosuppressants – the only approved nonsteroidal immunosuppressant in the setting of myasthenia gravis is azathioprine (AZA) – and corticosteroids
- In severe or critical courses:
- Plasma exchange
- I.v. immunoglobulins (IVIG)-use primarily in myasthenic crisis and in patients prior to thymectomy who do not respond to drug therapy; ventilation time may be shortened with IVIG use
- Cholinesterase inhibitors (cholinesterase inhibitors) – symptomatic therapy: improvement of neuromuscular excitation transmission by inhibiting the enzyme acetylcholinesterase → degradation of acetylcholine in the synaptic cleft is prevented → concentration and temporal availability of acetylcholine in the synaptic cleft increases → acetylcholine receptors are occupied longer and remain activated.
- Oral administration
- Pyridostigmine bromide – drug of choice for long-term oral treatment.
- In case of bromine intolerance, allergy, or hypersensitivity to bromide-containing preparations (rare): ambenonium chloride.
- Intravenous administration (agent of choice): neostigin and pyridostigmine.
- Oral administration
- Myasthenic crisis → intensive medical treatment.
- Hemapheresis procedure (procedure for extracorporeal apparative separation of blood with targeted extraction or removal of blood components).
- Plasmapheresis – therapeutic procedure for the effective removal of unwanted antibodies.
- Immunoadsorption – therapeutic procedure for the removal of autoantibodies and immune complexes in autoimmune diseases using an adsorption system.
- Intravenous immunoglobulins (IVIG).
- Methylprednisolone (pulse therapy).
- Hemapheresis procedure (procedure for extracorporeal apparative separation of blood with targeted extraction or removal of blood components).
- Therapy-refractory generalized acetylcholine receptor antibody-positive myasthenia gravis* : Rituximab (monoclonal antibody (IgG-1-kappa immunoglobulin) directed against surface antigen CD20); eculizumab (monoclonal antibody directed against complement factor C5).
- Thymectomy (surgical removal of the thymus gland; see “Surgical therapy” below).
- If sudden deterioration occurs in patients who initially responded well to treatment, cholinesterase inhibitors should be discontinued for several days and respiratory support provided.
* Failure to respond (“supoptimal or nonresponse”) and, on the other hand, intolerable side effects to previous standard and extended therapies according to current relevant guidelines.
