Acquired myasthenia gravis

Immunopathology

David P Richman, M. A. Agius

Research output: Contribution to journalArticle

18 Citations (Scopus)

Abstract

Much of the remarkable advance in our understanding of the immunopathology of MG relates to the availability of two gifts of nature that permit the ready purification of the antigen, AChR. Immunization of experimental animals with AChR has led to the development of the extremely faithful animal model, EAMG. Analysis of both EAMG and MG has revealed that the effector agents in this autoimmune disease are anti-AChR antibodies, whose production is regulated by anti-AChR CD4+ T cells. The pathogenic effects on neuromuscular transmission are mediated by antibody-induced antigenic modulation of end- plate AChR, end-plate membrane destruction through complement fixation and recruitment of inflammatory cells, and antibody-induced blockade of the function of the remaining AChR molecules. The origin of MG remains unknown. One theory proposes that dysregulation of the thymic control of tolerance plays an important role. An alternative hypothesis is that tolerance is broken as the result of a vigorous immune response directed against an invading microorganism that expresses a molecule that is similar to AChR, so- called molecular mimicry. This 'normal' response eventually cross-reacts with self-AChR, resulting in the autoimmune damage. Our current knowledge of MG has suggested a number of possible sites of therapeutic intervention that are under active study. Future information concerning the origin of the disease will likely be useful in the design of more effective treatment for this and other related autoimmune diseases.

Original languageEnglish (US)
Pages (from-to)273-284
Number of pages12
JournalNeurologic Clinics
Volume12
Issue number2
StatePublished - 1994

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Myasthenia Gravis
Autoimmune Diseases
Antigenic Modulation
Molecular Mimicry
Gift Giving
Antibodies
Antibody Formation
Anti-Idiotypic Antibodies
Immunization
Animal Models
T-Lymphocytes
Antigens
Membranes
Therapeutics

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Acquired myasthenia gravis : Immunopathology. / Richman, David P; Agius, M. A.

In: Neurologic Clinics, Vol. 12, No. 2, 1994, p. 273-284.

Research output: Contribution to journalArticle

Richman, DP & Agius, MA 1994, 'Acquired myasthenia gravis: Immunopathology', Neurologic Clinics, vol. 12, no. 2, pp. 273-284.
Richman, David P ; Agius, M. A. / Acquired myasthenia gravis : Immunopathology. In: Neurologic Clinics. 1994 ; Vol. 12, No. 2. pp. 273-284.
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