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Udgivet

Chylomicronemia from GPIHBP1 autoantibodies

Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

DOI

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  2. Chylomicronemia From GPIHBP1 Autoantibodies Successfully Treated With Rituximab: A Case Report

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  3. The structural basis for monoclonal antibody 5D2 binding to the tryptophan-rich loop of lipoprotein lipase

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  • Kazuya Miyashita
  • Jens Lutz
  • Lisa C Hudgins
  • Dana Toib
  • Ambika P Ashraf
  • Wenxin Song
  • Masami Murakami
  • Katsuyuki Nakajima
  • Michael Ploug
  • Loren G Fong
  • Stephen G Young
  • Anne P Beigneux
Vis graf over relationer

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.

OriginalsprogEngelsk
TidsskriftJournal of Lipid Research
ISSN0022-2275
DOI
StatusUdgivet - 18 sep. 2020

Bibliografisk note

Published under license by The American Society for Biochemistry and Molecular Biology, Inc.

ID: 61071132