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Review
. 2012 Jul;8(5):427-38.
doi: 10.1586/eci.12.34.

Muscle autoantibodies in myasthenia gravis: beyond diagnosis?

Matthew N Meriggioli  1 , Donald B Sanders
Affiliations
Review

Muscle autoantibodies in myasthenia gravis: beyond diagnosis?

Matthew N Meriggioli et al. Expert Rev Clin Immunol. 2012 Jul.

Abstract

Myasthenia gravis is an autoimmune disorder of the neuromuscular junction. A number of molecules, including ion channels and other proteins at the neuromuscular junction, may be targeted by autoantibodies leading to abnormal neuromuscular transmission. In approximately 85% of patients, autoantibodies, directed against the postsynaptic nicotinic acetylcholine receptor can be detected in the serum and confirm the diagnosis, but in general, do not precisely predict the degree of weakness or response to therapy. Antibodies to the muscle-specific tyrosine kinase are detected in approximately 50% of generalized myasthenia gravis patients who are seronegative for anti-acetylcholine receptor antibodies, and levels of anti-muscle-specific tyrosine kinase antibodies do appear to correlate with disease severity and treatment response. Antibodies to other muscle antigens may be found in the subsets of myasthenia gravis patients, potentially providing clinically useful diagnostic information, but their utility as relevant biomarkers (measures of disease state or response to treatment) is currently unclear.

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Figures

Figure 1
Figure 1. Muscle autoantigens in myasthenia gravis
The major pathologic antibodies in myasthenia gravis target AChR and MuSK on the postsynaptic membrane. Autoantibodies may also target other end-plate proteins (LRP4, rapsyn) and proteins involved in muscle contraction and excitation–contraction coupling (titin, myofibrillar proteins and RyR). ACh: Acetylcholine; AChR: Acetylcholine receptor; ColQ: Collagen Q; LRP4: Lipoprotein-related protein receptor 4; MuSK: Muscle-Specific tyrosine kinase; RyR: Ryanodine receptor.
Figure 2
Figure 2. Maximum acetylcholine receptor antibody levels in 865 patients with seropositive myasthenia gravis versus maximum class
ACh-Ab: Acetylcholine antibody; SE: Standard error. Reproduced with permission from [Sanders DB et al., Unpublished Data] © DB Sanders (2012).
Figure 3
Figure 3. Anti-acetylcholine receptor antibody levels in 120 myasthenia gravis patients grouped according to treatment response as assessed by the Myasthenia Gravis Foundation of America postintervention status scale
All AChR-Ab values; n = 120. AChR-Ab: Acetylcholine receptor antibody; IMP: Improved; MG: Myasthenia gravis; SE: Standard error; UNC: Unchanged; WORSE: Patient worsened. Reproduced with permission from [Sanders DB et al., Unpublished Data]. © DB Sanders (2012).
Figure 4
Figure 4. Distribution of diagnostic autoantibody results in myasthenia gravis
AChR: Acetylcholine receptor; LRP4: Lipoprotein-related protein receptor 4; MuSK: Muscle-specific tyrosine kinase.
See this image and copyright information in PMC

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