HLA class I and II in Lambert-Eaton myasthenic syndrome without associated tumor

P. W. Wirtz, B. O. Roep, G. M. Schreuder, P. A. van Doorn, B. G. van Engelen, J. B. Kuks, A. Twijnstra, M. de Visser, L. H. Visser, J. H. Wokke, A. R. Wintzen, J. J. Verschuuren

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Abstract

Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disorder, in which antibodies against voltage-gated calcium channels located at nerve terminals cause muscle weakness and autonomic dysfunction. In approximately half of the patients the autoimmune process is initiated by a tumor. In the other half of patients no tumor is found and the etiology is unknown. The aims of this study were to investigate the strength of HLA-associations with nontumor LEMS (NT-LEMS) and to study the relation of HLA-haplotypes with age at onset of LEMS and other clinical features. Therefore, typing of HLA class I and II was performed in 19 patients with NT-LEMS, who were clinically evaluated. NT-LEMS was significantly associated with alleles of both HLA-class I (i.e. HLA-B8) as well as -class II (i.e. HLA-DR3 and -DQ2). HLA-B8+ patients had significantly younger age at onset of LEMS and tended to be female. This study shows that HLA-class I haplotype is associated with a distinct phenotype in NT-LEMS
Original languageEnglish
Pages (from-to)809-813
JournalHuman immunology
Volume62
Issue number8
DOIs
Publication statusPublished - 2001

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