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Chylomicronemia from GPIHBP1 autoantibodies.
Journal of Lipid Research ( IF 6.5 ) Pub Date : 2020-09-18 , DOI: 10.1194/jlr.r120001116
Kazuya Miyashita 1, 2 , Jens Lutz 3 , Lisa C Hudgins 4 , Dana Toib 5, 6 , Ambika P Ashraf 7 , Wenxin Song 8 , Masami Murakami 1 , Katsuyuki Nakajima 1 , Michael Ploug 9, 10 , Loren G Fong 11 , Stephen G Young 11, 12 , Anne P Beigneux 11
Affiliation  

Some cases of chylomicronemia are caused by autoantibodies against GPIHBP1, an endothelial cell protein that shuttles lipoprotein lipase (LPL) to the capillary lumen. GPIHBP1 autoantibodies prevent binding and transport of LPL by GPIHBP1, thereby disrupting the lipolytic processing of triglyceride-rich lipoproteins. Here, we review the "GPIHBP1 autoantibody syndrome" and summarize clinical and laboratory findings in 22 patients. All patients had GPIHBP1 autoantibodies and chylomicronemia, but we did not find a correlation between triglyceride levels and autoantibody levels. Many of the patients had a history of pancreatitis, and most had clinical and/or serological evidence of autoimmune disease. IgA autoantibodies were present in all patients, and IgG4 autoantibodies were present in 19 of 22 patients. Patients with GPIHBP1 autoantibodies had low plasma LPL levels, consistent with impaired delivery of LPL into capillaries. Plasma levels of GPIHBP1, measured with a monoclonal antibody-based ELISA, were very low in 17 patients, reflecting the inability of the ELISA to detect GPIHBP1 in the presence of autoantibodies (immunoassay interference). However, GPIHBP1 levels were very high in 5 patients, indicating little capacity of their autoantibodies to interfere with the ELISA. Recently, several GPIHBP1 autoantibody syndrome patients were treated successfully with rituximab, resulting in the disappearance of GPIHBP1 autoantibodies and normalization of both plasma triglyceride and LPL levels. The GPIHBP1 autoantibody syndrome should be considered in any patient with newly acquired and unexplained chylomicronemia.

中文翻译:

GPIHBP1 自身抗体引起的乳糜微粒血症。

一些乳糜微粒血症病例是由针对 GPIHBP1 的自身抗体引起的,GPIHBP1 是一种将脂蛋白脂肪酶 (LPL) 运送到毛细血管腔的内皮细胞蛋白。GPIHBP1 自身抗体可阻止 GPIHBP1 与 LPL 的结合和转运,从而破坏富含甘油三酯的脂蛋白的脂肪分解过程。在此,我们回顾了“GPIHBP1 自身抗体综合征”并总结了 22 名患者的临床和实验室检查结果。所有患者都有 GPIHBP1 自身抗体和乳糜微粒血症,但我们没有发现甘油三酯水平和自身抗体水平之间的相关性。许多患者有胰腺炎病史,大多数有自身免疫性疾病的临床和/或血清学证据。所有患者均存在 IgA 自身抗体,22 名患者中有 19 名存在 IgG4 自身抗体。携带 GPIHBP1 自身抗体的患者血浆 LPL 水平较低,这与 LPL 进入毛细血管的输送受损一致。使用基于单克隆抗体的 ELISA 测量,17 名患者的 GPIHBP1 血浆水平非常低,这反映出 ELISA 无法在自身抗体存在的情况下检测 GPIHBP1(免疫测定干扰)。然而,5 名患者的 GPIHBP1 水平非常高,表明他们的自身抗体干扰 ELISA 的能力很小。最近,几位GPIHBP1自身抗体综合征患者接受了利妥昔单抗的成功治疗,导致GPIHBP1自身抗体消失,血浆甘油三酯和LPL水平恢复正常。对于任何新出现且无法解释的乳糜微粒血症患者,均应考虑 GPIHBP1 自身抗体综合征。
更新日期:2020-09-21
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