Center for Neuromuscular Diseases, Unit of Neurology, ASST "Spedali Civili" and University of Brescia, Italy.
Laboratorio Istocompatibilità, Servizio di Immunoematologia e Medicina Trasfusionale SIMT, ASST "Spedali Civili", Brescia, Italy.
J Neurol Sci. 2019 Dec 15;407:116533. doi: 10.1016/j.jns.2019.116533. Epub 2019 Oct 23.
CIDP spectrum encompasses several clinical variants and the reasons of the heterogeneous clinical expression and the variable response to therapy are scarcely known. HLA associations are common in dysimmune conditions. In CIDP, few studies reported no associations or HLA-DR13/DQ6 association in some populations but, to date, a clear confirmed association is lacking. We analyzed expression of HLA-DR and DQ haplotypes in 24 CIDP patients and 216 healthy subject. HLA-DR3 and DR3/DQ2 were significantly more frequent in CIDP patients than in the control group. The DR3 and DR3/DQ2 positive patients present with more frequent relapsing course, worse response to IVIg, higher inflammatory neuropathy sensory sumscore (ISS) and Rotterdam Inflammatory Neuropathy Cause and Treatment Scale (INCAT) than negative patients.
CIDP 谱涵盖几种临床变异，导致其临床表现异质性和对治疗反应不同的原因尚不清楚。HLA 相关性在自身免疫性疾病中很常见。在 CIDP 中，一些研究报告在某些人群中无相关性或 HLA-DR13/DQ6 相关性，但迄今为止，尚无明确的相关性被证实。我们分析了 24 例 CIDP 患者和 216 例健康对照者的 HLA-DR 和 DQ 单倍型表达。与对照组相比，HLA-DR3 和 DR3/DQ2 在 CIDP 患者中更为常见。DR3 和 DR3/DQ2 阳性患者的复发病程更频繁，对 IVIg 的反应更差，炎症性神经病感觉总评分（ISS）和鹿特丹炎症性神经病病因和治疗量表（INCAT）更高。
