Antibodies to myelin-associated glycoprotein (anti-MAG) in IgM amyloidosis may influence expression of neuropathy in rare patients
Antibodies to myelin-associated glycoprotein (anti-MAG) in IgM amyloidosis may influence expression of neuropathy in rare patients
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DOI：
10.1002/mus.20955
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发表时间：
2008-04-01
期刊：
影响因子：
3.4
通讯作者：
Klein, Christopher J.
中科院分区：
文献类型：
--
作者：
Garces-Sanchez, Mercedes;Dyck, Peter J.;Klein, Christopher J.
我们研究了髓鞘相关糖蛋白抗体（抗MAG）是否影响原发性（AL IgM）淀粉样变性的神经病变发生和表型。对46例IgM型淀粉样变性患者（21例伴多发性神经病）和21例无神经病的IgM型MGUS（意义不明的单克隆丙种球蛋白病）对照进行了抗MAG和交叉反应的磺基葡萄糖醛酸副糖苷抗体（SGPG）研究。我们评估了神经病变的发生、表型和神经传导的属性及其与抗体活性的关系。46例IgM型淀粉样变性患者中有20例（7例伴多发性神经病，13例不伴多发性神经病）经酶联免疫吸附试验（ELISA）检测抗MAG或SGPG升高。2例IgM型淀粉样变性多发性神经病患者免疫印迹法（WB）检测MAG抗体阳性。其中一名患者，具有最高的抗MAG滴度，具有疼痛的感觉性共济失调，伴有明显的脱髓鞘和腓肠神经中的淀粉样蛋白沉积。另一个抗MAG WB阳性淀粉样蛋白患者有轴突神经病变和自主神经功能障碍。在21例IgM MGUS对照中，有12例无神经病变（平均随访时间为11年），抗MAG抗体水平较低。我们的结论是，发现血清抗MAG抗体并不能排除原发性淀粉样变性的诊断。它们似乎不影响多发性神经病的发生或表达，除了可能在WB阳性的偶尔病例中。
We have examined whether antibodies to myelin-associated glycoprotein (anti-MAG) influence neuropathy occurrence and phenotype in primary (AL IgM) amyloidosis. Anti-MAG and the cross-reacted sulfoglucuronyl paragloboside antibodies (SGPG) were studied in 46 patients with IgM amyloidosis (21 with polyneuropathy), and 21 matched IgM MGUS (monoclonal gammopathies of undetermined significance) controls without neuropathy. We assessed the occurrence, phenotype of neuropathy, and attributes of nerve conduction and their relation to antibody activity. Twenty of 46 patients with IgM amyloidosis (7 with and 13 without polyneuropathy) had elevation of anti-MAG or SGPG by enzyme-linked immunosorbent assay (ELISA). Two of the polyneuropathy patients with IgM amyloidosis had antibodies to MAG based on Western blot (WB) positivity. One of these patients, with the highest anti-MAG titer, had a painful sensory ataxia, with prominent demyelination, and amyloid deposition in sural nerve. The other anti-MAG WB-positive amyloid patient had an axonal neuropathy and dysautonomia. Low levels of anti-MAG antibodies were found in 12 of 21 IgM MGUS controls without neuropathy (mean follow-up, 11 years). We conclude that finding serum anti-MAG antibodies does not exclude the diagnosis of primary amyloidosis. They do not appear to affect the occurrence or expression of polyneuropathy, except possibly in occasional cases with WB positivity.