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Three types of striational antibodies in myasthenia gravis.

Suzuki S, Utsugisawa K, Nagane Y, Suzuki N - Autoimmune Dis (2011)

Bottom Line: Striational antibodies, which react with epitopes on the muscle proteins titin, ryanodine receptor (RyR), and Kv1.4, are frequently found in MG patients with late-onset and thymoma.The presence of striational antibodies is associated with more severe disease in all MG subgroups.Detection of striational antibodies provides more specific and useful clinical information in MG patients.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan.

ABSTRACT
Myasthenia gravis (MG) is caused by antibodies that react mainly with the acetylcholine receptor on the postsynaptic site of the neuromuscular junction. A wide range of clinical presentations and associated features allow MG to be classified into subtypes based on autoantibody status. Striational antibodies, which react with epitopes on the muscle proteins titin, ryanodine receptor (RyR), and Kv1.4, are frequently found in MG patients with late-onset and thymoma. Antititin and anti-RyR antibodies are determined by enzyme-linked immunosorbent assay or immunoblot. More recently, a method for the detection of anti-Kv1.4 autoantibodies has become available, involving 12-15% of all MG patients. The presence of striational antibodies is associated with more severe disease in all MG subgroups. Anti-Kv1.4 antibody is a useful marker for the potential development of lethal autoimmune myocarditis and response to calcineurin inhibitors. Detection of striational antibodies provides more specific and useful clinical information in MG patients.

No MeSH data available.


Related in: MedlinePlus

Electrocardiogram in myasthenia gravis patients with anti-Kv1.4 antibodies. (a) Ventricular tachycardia, (b) sick sinus syndrome, and (c) complete atrial ventricular block.
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fig1: Electrocardiogram in myasthenia gravis patients with anti-Kv1.4 antibodies. (a) Ventricular tachycardia, (b) sick sinus syndrome, and (c) complete atrial ventricular block.

Mentions: We also confirmed that some MG patients with anti-Kv1.4 antibodies had a risk for lethal arrhythmias including ventricular tachycardia, sick sinus syndrome, and complete atrial ventricular block (Figure 1) [48, 49]. We emphasize that special attention should be paid to MG patients with anti-Kv1.4 antibodies. Although various Kv channels are expressed in cardiac muscles and implicated in electrophysiological function, the association between autoantibodies to Kv channels and arrhythmias is not fully elucidated [50]. We are now investigating the mechanisms of the cardiac involvement of Kv1.4 autoimmunity.


Three types of striational antibodies in myasthenia gravis.

Suzuki S, Utsugisawa K, Nagane Y, Suzuki N - Autoimmune Dis (2011)

Electrocardiogram in myasthenia gravis patients with anti-Kv1.4 antibodies. (a) Ventricular tachycardia, (b) sick sinus syndrome, and (c) complete atrial ventricular block.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3139883&req=5

fig1: Electrocardiogram in myasthenia gravis patients with anti-Kv1.4 antibodies. (a) Ventricular tachycardia, (b) sick sinus syndrome, and (c) complete atrial ventricular block.
Mentions: We also confirmed that some MG patients with anti-Kv1.4 antibodies had a risk for lethal arrhythmias including ventricular tachycardia, sick sinus syndrome, and complete atrial ventricular block (Figure 1) [48, 49]. We emphasize that special attention should be paid to MG patients with anti-Kv1.4 antibodies. Although various Kv channels are expressed in cardiac muscles and implicated in electrophysiological function, the association between autoantibodies to Kv channels and arrhythmias is not fully elucidated [50]. We are now investigating the mechanisms of the cardiac involvement of Kv1.4 autoimmunity.

Bottom Line: Striational antibodies, which react with epitopes on the muscle proteins titin, ryanodine receptor (RyR), and Kv1.4, are frequently found in MG patients with late-onset and thymoma.The presence of striational antibodies is associated with more severe disease in all MG subgroups.Detection of striational antibodies provides more specific and useful clinical information in MG patients.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan.

ABSTRACT
Myasthenia gravis (MG) is caused by antibodies that react mainly with the acetylcholine receptor on the postsynaptic site of the neuromuscular junction. A wide range of clinical presentations and associated features allow MG to be classified into subtypes based on autoantibody status. Striational antibodies, which react with epitopes on the muscle proteins titin, ryanodine receptor (RyR), and Kv1.4, are frequently found in MG patients with late-onset and thymoma. Antititin and anti-RyR antibodies are determined by enzyme-linked immunosorbent assay or immunoblot. More recently, a method for the detection of anti-Kv1.4 autoantibodies has become available, involving 12-15% of all MG patients. The presence of striational antibodies is associated with more severe disease in all MG subgroups. Anti-Kv1.4 antibody is a useful marker for the potential development of lethal autoimmune myocarditis and response to calcineurin inhibitors. Detection of striational antibodies provides more specific and useful clinical information in MG patients.

No MeSH data available.


Related in: MedlinePlus