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肾病综合征、单克隆丙种球蛋白病和自身免疫性高脂血症。

Nephrotic syndrome, monoclonal gammopathy and auto-immune hyperlipidaemia.

作者信息

Beaumont J L, Antonucci M, Lagrue G, Guedon J, Perol R

出版信息

Clin Exp Immunol. 1974 Oct;18(2):225-36.

Abstract

Hyperlipidaemia, glomerulonephritis and IgG λ monoclonal gammopathy were found to be associated in two patients (Ler. and Lac.). A severe atherosclerosis was also present in the former patient. After a purification–activation procedure active IgG λ was obtained from the serum chylomicra and from the ammonium sulphate-precipitated serum gamma-globulins. In passive haemagglutination tests, including inhibition tests, the IgG λ Ler. reacted specifically with the protein moiety of human low density lipoproteins (LDL) and the IgG λ Lac. reacted with human, rat and rabbit very low density lipoproteins (VLDL) as well as with human serum albumin (HSA). These IgG λ behaved like autoantibodies though no activity was found in whole serum, in which they are probably blocked by the corresponding antigens. They are very similar, although not identical, to the autoantibodies found in auto-immune hyperlipidaemia (AIH) and it is proposed that in these patients the hyperlipidaemia is a variety of AIH. Finally it is suggested that the hyperlipidaemia associated with glomerulonephritis chiefly in the nephrotic syndrome may sometimes be AIH. If this is the case the renal lesions and the hyperlipidaemia may be different expressions of an immune complex disease which may also lead to other tissue lesions such as atherosclerosis.

摘要

在两名患者(Ler.和Lac.)中发现高脂血症、肾小球肾炎和IgG λ单克隆丙种球蛋白病有关联。前一名患者还存在严重的动脉粥样硬化。经过纯化-激活程序后,从血清乳糜微粒和硫酸铵沉淀的血清γ-球蛋白中获得了活性IgG λ。在包括抑制试验在内的被动血凝试验中,IgG λ Ler.与人低密度脂蛋白(LDL)的蛋白质部分发生特异性反应,而IgG λ Lac.与人、大鼠和兔的极低密度脂蛋白(VLDL)以及人血清白蛋白(HSA)发生反应。这些IgG λ表现得像自身抗体,尽管在全血清中未发现活性,它们可能在全血清中被相应抗原所阻断。它们与自身免疫性高脂血症(AIH)中发现的自身抗体非常相似,尽管并不完全相同,并且有人提出在这些患者中高脂血症是AIH的一种类型。最后有人提出,主要在肾病综合征中与肾小球肾炎相关的高脂血症有时可能是AIH。如果是这样,肾脏病变和高脂血症可能是免疫复合物疾病的不同表现形式,这也可能导致其他组织病变,如动脉粥样硬化。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fa3e/1537892/5dc677140417/clinexpimmunol00267-0062-a.jpg

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