[Auto-antibodies against acetylcholine receptors in myasthenia gravis].
USEFULLNESS OF DETECTION OF ANTIBODIES AGAINST THE ACETYLCHOLINE RECEPTOR IN THE DIAGNOSIS OF MYASTHENIA GRAVIS (MG): MG is an autoimmune disorder including a fatigability of skeletal muscles and the presence of antibodies against the acetylcholine receptor (AChRAbs). These Abs are pathogenic by blocking the acetylcholine receptor within the neuromuscular junction. A transient neonatal MG occurs in 12% of babies born to myasthenic mothers. AChRAbs have been found in 77 to 89% of systemic MG and in 47 to 60% in ocular form of MG. These Abs are generally directed against the "Main Immunogenic Region" (MIR) within the a subunit of the receptor; others are directed against beta, gamma and epsilon subunits. Eleven to 23% of the MG are negative for the Abs directed against the a subunit reinforcing the interest to detect the other AChRAbs, specially the anti-epsilon subunit Abs and the Abs directed against the muscle-specific receptor tyrosine kinase. MULTICENTER EVALUATION: In order to compare the results of AChRAbs for a same patient from various laboratories, a multicenter study was performed on 26 sera with different titers of AChRAbs using radioimmunoassays. Six french laboratories have participated to this study. The antigen was obtained from TE671 cells for 5 laboratories (3 used a commercial test) or from human muscle (1 laboratory). At the end of the study, the qualitative analysis of the results showed a concordance of 92.3%. The interpretation of AChRAbs assays can thus be done by clinicians whatever the test is used. A same quality control is nowadays used by the six laboratories in order to estimate quantitative results according to the assay performed. USEFULNESS OF THE OTHER ABS: The Abs directed against the striated muscle are good markers of thymoma in MG but are not specific for MG. The Abs directed against titin which is the major target of the anti-striated muscle Abs, are also good biological markers of thymoma specially in MG adults younger than 60 years. The detection of anti-myoid cells and thymic reticuloepithelial cells Abs is no more useful due to a lower sensitivity compared to the Abs directed against the striated muscle.
Fabien
N
N
Laboratoire d'Auto-immunité, Service d'immunologie, Centre Hospitalier Lyon-Sud, F69495 Pierre-Bénite. nicole.fabien@chu-lyon.fr
Huchet
F X
FX
Sanmarco
M
M
Dubucquoi
S
S
Wagner
S
S
Yamamoto
A M
AM
D001323:Autoantibodies / Q000032:analysis* / Q000276:immunology; D015415:Biomarkers / Q000032:analysis; D006801:Humans; D009157:Myasthenia Gravis / Q000175:diagnosis* / Q000276:immunology*; D015588:Observer Variation; D011786:Quality Control; D011863:Radioimmunoassay; D011950:Receptors, Cholinergic / Q000276:immunology*; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity
D001323:Autoantibodies; D015415:Biomarkers; D011950:Receptors, Cholinergic