Clinical fluctuations in MuSK myasthenia gravis are related to antigen-specific IgG4 instead fo IgG1

被引:109
作者
Niks, E. H. [1 ]
van Leeuwen, Y. [2 ]
Leite, M. I. [3 ]
Dekker, F. W. [2 ]
Wintzen, A. R. [1 ]
Wirtz, P. W. [5 ]
Vincent, A. [3 ]
van Tol, M. J. D. [4 ]
Zijde, C. M. Jol-van der [4 ]
Verschuuren, J. J. G. M. [1 ]
机构
[1] Leiden Univ, Med Ctr, Dept Neurol, NL-2300 RC Leiden, Netherlands
[2] Leiden Univ, Med Ctr, Dept Clin Epidemiol, NL-2300 RC Leiden, Netherlands
[3] Univ Oxford, John Radcliffe Hosp, Weatherall Inst Mol Med, Dept Clin Neurol,Neurosci Grp, Oxford OX3 9DS, England
[4] Leiden Univ, Med Ctr, Dept Pediat, Sect Immunol Hematol Oncol Bone Marrow Transplant, NL-2300 RC Leiden, Netherlands
[5] Haga Hosp, Dept Neurol, NL-2504 LN The Hague, Netherlands
关键词
antibodies; IgG4; immunoglobulin isotypes; myasthenia gravis; MuSK; seronegative;
D O I
10.1016/j.jneuroim.2008.01.013
中图分类号
R392 [医学免疫学]; Q939.91 [免疫学];
学科分类号
100102 ;
摘要
We studied the longitudinal relation between disease severity and titers of antigen-specific IgG subclasses in sera of patients with myasthenia gravis and antibodies to Muscle Specific Kinase (MuSK MG). Six patients were included of whom 55 samples had been collected during 2.5-13.4 years. Anti-MuSK antibodies were determined by ELISA and with a cell-based immunofluorescence assay. Disease severity was scored on a semi Continuous scale. Only antigen-specific IgG4, and not IgG 1, titers were significantly associated with disease severity in a linear mixed effect model (p = 0.036). Levels of IgG4 antibodies were above IgG I in all samples except in one patient who went into clinical remission while switching from IgG4 to IgG1. The results support an important role for IgG4 in the pathogenesis of MuSK MG, in contrast to MG with anti-acetylcholine receptor antibodies. (C) 2008 Elsevier B.V. All rights reserved.
引用
收藏
页码:151 / 156
页数:6
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