Higher frequencies of HLA DQB1*05:01 and anti-glycosphingolipid antibodies in a cluster of severe Guillain-Barr, syndrome
Higher frequencies of HLA DQB1*05:01 and anti-glycosphingolipid antibodies in a cluster of severe Guillain-Barr, syndrome
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DOI：
10.1007/s00415-016-8237-6
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发表时间：
2016-10-01
影响因子：
6
通讯作者：
Hemmer, Bernhard
中科院分区：
文献类型：
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作者：
Schirmer, L.;Worthington, V.;Hemmer, Bernhard
到目前为止，很少有区域性和季节性格林-巴利综合征（GBS）集群的报告。目前尚不清楚散发性GBS患者在临床和临床旁参数、HLA相关性和对鞘糖脂和空肠弯曲菌（Cj）的抗体应答方面是否与GBS集群不同。我们研究了来自德国大慕尼黑地区的40例GBS患者，其中14例在2010年秋季3个月内入院，定义了一组GBS。对HLA基因DRB 1、DQB 1和DPB 1进行基于测序的HLA分型，并进行抗鞘糖脂抗体的ELISA。获得并分析了临床和副临床结果（Cj血清反应性、脑脊液参数和电生理学）。与散发性GBS病例（13%，p = 0.017）相比，GBS集群患者的特征在于更严重的临床表型，更多患者需要机械通气，以及针对硫苷脂、GalC和某些神经节苷脂表位的自身抗体的频率更高（54%）。与散发病例（46%，p = 0.155）相比，GBS集群患者（69%）中的Cj血清阳性倾向于更高。我们注意到，与散发性GBS患者（3%，p = 0.019）相比，在群集队列（23%）中HLA II类等位基因DQB 1 *05：01的频率更高。严重GBS的集群定义为较高频率的抗鞘糖脂自身抗体。HLA Ⅱ类等位基因DQB 1 *05：01可能导致聚集性患者的临床恶化。
Few regional and seasonal Guillain-Barr, syndrome (GBS) clusters have been reported so far. It is unknown whether patients suffering from sporadic GBS differ from GBS clusters with respect to clinical and paraclinical parameters, HLA association and antibody response to glycosphingolipids and Campylobacter jejuni (Cj). We examined 40 consecutive patients with GBS from the greater Munich area in Germany with 14 of those admitted within a period of 3 months in fall 2010 defining a cluster of GBS. Sequencing-based HLA typing of the HLA genes DRB1, DQB1, and DPB1 was performed, and ELISA for anti-glycosphingolipid antibodies was carried out. Clinical and paraclinical findings (Cj seroreactivity, cerebrospinal fluid parameters, and electrophysiology) were obtained and analyzed. GBS cluster patients were characterized by a more severe clinical phenotype with more patients requiring mechanical ventilation and higher frequencies of autoantibodies against sulfatide, GalC and certain ganglioside epitopes (54 %) as compared to sporadic GBS cases (13 %, p = 0.017). Cj seropositivity tended to be higher within GBS cluster patients (69 %) as compared to sporadic cases (46 %, p = 0.155). We noted higher frequencies of HLA class II allele DQB1*05:01 in the cluster cohort (23 %) as compared to sporadic GBS patients (3 %, p = 0.019). Cluster of severe GBS was defined by higher frequencies of autoantibodies against glycosphingolipids. HLA class II allele DQB1*05:01 might contribute to clinical worsening in the cluster patients.