Autoimmunity related to IgM monoclonal gammopathy of undetermined significance. Peripheral neuropathy and connective tissue sensibilization caused by IgM M-proteins.

Autoimmunity related to IgM monoclonal gammopathy of undetermined significance. Peripheral neuropathy and connective tissue sensibilization caused by IgM M-proteins.
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与 IgM 单克隆丙种球蛋白病相关的自身免疫具有未确定的意义。

DOI:
10.1111/j.0954-6820.1988.tb15795.x
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发表时间:
2009
影响因子:
--
通讯作者:
E. Hippe
E. Hippe
中科院分区:
--
文献类型:
--
作者:
V. Jønsson;H. Schrøder;T. Staehelin Jensen;C. Nolsøe;B. Stigsby;W. Trojaborg;A. Svejgaard;E. Hippe

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在10个连续的IgM单克隆丙种球蛋白病的意义不明(MGUS)的情况下,8 M-蛋白对各种组织的直接和间接的免疫荧光研究皮肤和/或腓肠神经活检估计的特异性。其中5例有神经病变。在其中三个,包括两个兄弟姐妹与脱髓鞘周围神经病变,IgM结合髓鞘相关糖蛋白(MAG)的周围神经。一个有轴突神经病变与IgM抗体活性对神经鞘和神经内膜,而另一个感染后神经炎的IgM抗体活性对结构的神经内膜,但没有IgM抗体自身免疫的直接荧光试验。后者在临床上的改善与M蛋白的减少平行,表明自身抗体的致病作用。在另外三个病例中,IgM与结缔组织结构结合,其中两个病例在间接荧光试验中也有针对神经鞘和神经内膜的血浆抗体。最后,2例显示M蛋白对任何组织结构无反应。由于自身免疫性发病机制被怀疑,HLA型7例患者的报告。
In eight of 10 consecutive cases of IgM monoclonal gammopathy of undetermined significance (MGUS), the M-protein had specificity towards various tissues as estimated by direct and indirect immunofluorescence studies of skin and/or sural nerve biopsies. Five of the cases had neuropathy. In three of them, including two siblings with a demyelinating peripheral neuropathy, the IgM was bound to the myelin-associated glycoprotein (MAG) of peripheral nerves. One had axonal neuropathy with IgM activity against the peri- and endoneurium, while another case with post-infectious neuritis had IgM activity against structures in the endoneurium but no IgM autoimmunity in the direct fluorescence test. The latter improved clinically in parallel with a decrease in the M-protein indicating a pathogenetic role of the autoantibody. In three other cases, the IgM was bound to connective tissue structures, two of them also had plasma antibodies against the peri- and endoneurium in the indirect fluorescence test. Finally, two cases showed no reaction of the M-protein against any tissue structures. Since an autoimmune pathogenesis is suspected, the HLA types of seven patients are reported.
意义未明的单克隆丙种球蛋白病(MGUS):综述。
DOI: --
发表时间: 1982
期刊: Clinics in haematology
影响因子: --
作者:
Kyle,RA
通讯作者: Kyle,RA