Advances and ongoing research in the treatment of autoimmune neuromuscular junction disorders

Myasthenia gravis and Lambert-Eaton myasthenic syndrome are antibody-mediated autoimmune diseases of the neuromuscular junction that usually present with weakness in ocular muscles and in proximal muscles of the limb and trunk. Prognosis regarding muscle strength, functional abilities, quality of li...

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Bibliographic Details
Published in:Lancet neurology 2022-02, Vol.21 (2), p.189-202
Main Authors: Verschuuren, Jan JGM, Palace, Jacqueline, Murai, Hiroyuki, Tannemaat, Martijn R, Kaminski, Henry J, Bril, Vera
Format: Article
Language:English
Subjects:
Antibodies
Antigens
Autoimmune diseases
Autoimmune Diseases - therapy
Clinical trials
Corticosteroids
COVID-19
Disease
Drug dosages
Exercise
Humans
Immunoglobulins
Immunosuppressive agents
Immunotherapy
Lambert-Eaton myasthenic syndrome
Lambert-Eaton Myasthenic Syndrome - immunology
Lambert-Eaton Myasthenic Syndrome - therapy
Lymphocytes B
Medical research
Medical treatment
Muscle strength
Muscles
Myasthenia
Myasthenia gravis
Myasthenia Gravis - immunology
Myasthenia Gravis - therapy
Neuromuscular diseases
Neuromuscular Junction Diseases - immunology
Neuromuscular Junction Diseases - therapy
Neuromuscular junctions
Ocular muscles
Pandemics
Plasma
Quality of life
Remission (Medicine)
Steroids
Strength training
Vaccination
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Summary:Myasthenia gravis and Lambert-Eaton myasthenic syndrome are antibody-mediated autoimmune diseases of the neuromuscular junction that usually present with weakness in ocular muscles and in proximal muscles of the limb and trunk. Prognosis regarding muscle strength, functional abilities, quality of life, and survival is generally good. However, some patients do not respond to treatment. Symptomatic drugs, corticosteroids, and steroid-sparing immunosuppressive drugs remain the cornerstone of treatment. In the past few years, new biological agents against complement, the FcRn receptor, or B-cell antigens have been tested in clinical trials. These new therapies extend the possibilities for targeted immunotherapies and promise exciting new options with a relatively rapid mode of action. Challenges in their use might occur, with barriers due to an increase in cost of care and additional considerations in the choice of drugs, and potential consequences of infection and vaccination due to the COVID-19 pandemic.
ISSN:1474-4422
1474-4465
DOI:10.1016/S1474-4422(21)00463-4