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Journal article
Chylomicronemia from GPIHBP1 autoantibodies
Journal of lipid research, v 61(11), pp 1365-1376
Nov 2020
PMID: 32948662
Featured in Collection : UN Sustainable Development Goals @ Drexel
Abstract
Some cases of chylomicronemia are caused by autoantibodies against glycosylphosphatidylinositol-anchored HDL binding protein 1 (GPIHBP1), an endothelial cell protein that shuttles 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 five 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.
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Details
- Title
- Chylomicronemia from GPIHBP1 autoantibodies
- Creators
- Kazuya Miyashita - Gunma UniversityJens Lutz - Medical Clinic, Nephrology-Infectious Diseases, Central Rhine Hospital Group, Koblenz, Germany.Lisa C. Hudgins - The Rogosin InstituteDana Toib - Drexel UniversityAmbika P. Ashraf - University of Alabama at BirminghamWenxin Song - University of California, Los AngelesMasami Murakami - Gunma UniversityKatsuyuki Nakajima - Gunma UniversityMichael Ploug - Biotechnology Research Innovation Center, Copenhagen University, Copenhagen, DenmarkLoren G. Fong - University of California, Los AngelesStephen G. Young - University of California, Los AngelesAnne P. Beigneux - University of California, Los Angeles
- Publication Details
- Journal of lipid research, v 61(11), pp 1365-1376
- Publisher
- Elsevier
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- Pediatrics
- Web of Science ID
- WOS:000586417000004
- Scopus ID
- 2-s2.0-85094983510
- Other Identifier
- 991019168333604721
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- Collaboration types
- Industry collaboration
- Domestic collaboration
- International collaboration
- Web of Science research areas
- Biochemistry & Molecular Biology