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

Letters3 November 2020Chylomicronemia From GPIHBP1 Autoantibodies Successfully Treated With Rituximab: A Case ReportJens Lutz, MD, Malgorzata Dunaj-Kazmierowska, MD, Sven Arcan, MD, Ursula Kassner, MD, Kazuya Miyashita, BS, Masami Murakami, MD, Michael Ploug, PhD, Loren G. Fong, PhD, Stephen G. Young, MD, Katsuyuki Nakajima, PhD, and Anne P. Beigneux, PhDJens Lutz, MDCentral Rhine Hospital Group, Koblenz, Germany (J.L., M.D., S.A.), Malgorzata Dunaj-Kazmierowska, MDCentral Rhine Hospital Group, Koblenz, Germany (J.L., M.D., S.A.), Sven Arcan, MDCentral Rhine Hospital Group, Koblenz, Germany (J.L., M.D., S.A.), Ursula Kassner, MDCharité University Medicine Campus Virchow, Berlin, Germany (U.K.), Kazuya Miyashita, BSGunma University Graduate School of Medicine, Maebashi, Japan (K.M., M.M., K.N.), Masami Murakami, MDGunma University Graduate School of Medicine, Maebashi, Japan (K.M., M.M., K.N.), Michael Ploug, PhDFinsen Laboratory, Rigshospitalet, Copenhagen, Denmark (M.P.), Loren G. Fong, PhDUniversity of California, Los Angeles, Los Angeles, California (L.G.F., S.G.Y., A.P.B.), Stephen G. Young, MDUniversity of California, Los Angeles, Los Angeles, California (L.G.F., S.G.Y., A.P.B.), Katsuyuki Nakajima, PhDGunma University Graduate School of Medicine, Maebashi, Japan (K.M., M.M., K.N.), and Anne P. Beigneux, PhDUniversity of California, Los Angeles, Los Angeles, California (L.G.F., S.G.Y., A.P.B.)Author, Article, and Disclosure Informationhttps://doi.org/10.7326/L20-0327 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail Background: Autoantibodies against glycosylphosphatidylinositol-anchored high-density lipoprotein–binding protein 1 (GPIHBP1), a cell-surface protein of capillary endothelial cells, block GPIHBP1-mediated transport of lipoprotein lipase (LPL) to the capillary lumen, resulting in impaired processing of triglyceride-rich lipoproteins (that is, chylomicrons and very-low-density lipoprotein) and severe hypertriglyceridemia (chylomicronemia) (1–3). Chylomicronemia resulting from GPIHBP1 autoantibodies is known as the "GPIHBP1 autoantibody syndrome" (1), and little is known about how to treat it.Objective: To inform internal medicine specialists that some cases of newly acquired chylomicronemia are caused by GPIHBP1 autoantibodies and that this disorder can be successfully treated with rituximab (resulting in disappearance of GPIHBP1 ...References1. Beigneux AP, Miyashita K, Ploug M, et al. Autoantibodies against GPIHBP1 as a cause of hypertriglyceridemia. N Engl J Med. 2017;376:1647-1658. [PMID: 28402248] doi:10.1056/NEJMoa1611930 CrossrefMedlineGoogle Scholar2. Hu X, Dallinga-Thie GM, Hovingh GK, et al. GPIHBP1 autoantibodies in a patient with unexplained chylomicronemia. J Clin Lipidol. 2017;11:964-971. [PMID: 28666713] doi:10.1016/j.jacl.2017.05.017 CrossrefMedlineGoogle Scholar3. Eguchi J, Miyashita K, Fukamachi I, et al. GPIHBP1 autoantibody syndrome during interferon β1a treatment. J Clin Lipidol. 2019;13:62-69. [PMID: 30514621] doi:10.1016/j.jacl.2018.10.004 CrossrefMedlineGoogle Scholar4. Kaegi C, Wuest B, Schreiner J, et al. Systematic review of safety and efficacy of rituximab in treating immune-mediated disorders. Front Immunol. 2019;10:1990. [PMID: 31555262] doi:10.3389/fimmu.2019.01990 CrossrefMedlineGoogle Scholar5. Chait A, Eckel RH. The chylomicronemia syndrome is most often multifactorial: a narrative review of causes and treatment. Ann Intern Med. 2019;170:626-634. doi:10.7326/M19-0203 LinkGoogle Scholar Author, Article, and Disclosure InformationAffiliations: Central Rhine Hospital Group, Koblenz, Germany (J.L., M.D., S.A.)Charité University Medicine Campus Virchow, Berlin, Germany (U.K.)Gunma University Graduate School of Medicine, Maebashi, Japan (K.M., M.M., K.N.)Finsen Laboratory, Rigshospitalet, Copenhagen, Denmark (M.P.)University of California, Los Angeles, Los Angeles, California (L.G.F., S.G.Y., A.P.B.)Note: Drs. Lutz and Beigneux are equally responsible for this work.Acknowledgment: The authors thank Amira Abdelhamid, MD, and Miss Christine Sauer (Medical Clinic, Nephrology-Infectious Diseases, Central Rhine Hospital Group, Koblenz, Germany) for evaluating and treating the patient.Grant Support: By grants HL090553, HL087228, and HL125335 from the National Heart, Lung, and Blood Institute; Transatlantic Networks of Excellence grant 12CVD04 from the Leducq Foundation; and grant NNF17OC0026868 from the Novo Nordisk Foundation.Disclosures: Disclosures can be viewed at www.acponline.org/authors/icmje/ConflictOfInterestForms.do?msNum=L20-0327.Corresponding Authors: Jens Lutz, MD, Medical Clinic, Nephrology-Infectious Diseases, Gemeinschaftsklinikum Mittelrhein, Koblenzer Straße 115-155, 56073 Koblenz, Germany (e-mail, jens.[email protected]de); and Anne P. Beigneux, PhD, UCLA Department of Medicine, Division of Cardiology, 650 Charles E. Young Drive South, A2-237 CHS Building, Los Angeles, CA 90095 (e-mail, [email protected]ucla.edu).This article was published at Annals.org on 11 August 2020. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byAnti-GPIHBP1 Antibody-Positive Autoimmune Hyperchylomicronemia and Immune ThrombocytopeniaA case of hyperchylomicronemia associated with GPIHBP1 autoantibodies and fluctuating thyroid autoimmune diseaseA protein of capillary endothelial cells, GPIHBP1, is crucial for plasma triglyceride metabolismGPIHBP1 autoantibody is an independent risk factor for the recurrence of hypertriglyceridemia-induced acute pancreatitisGPIHBP1 and ANGPTL4 Utilize Protein Disorder to Orchestrate Order in Plasma Triglyceride Metabolism and Regulate Compartmentalization of LPL ActivityThe Importance of Lipoprotein Lipase Regulation in AtherosclerosisChylomicronemia from GPIHBP1 autoantibodiesA Comprehensive Update on the Chylomicronemia Syndrome 3 November 2020Volume 173, Issue 9Page: 764-765KeywordsAntibodiesAutoantibodiesAutoimmune diseasesBlood plasmaDisclosureDrugsLipidsLipoproteinsProteinsSerum triglycerides ePublished: 11 August 2020 Issue Published: 3 November 2020 Copyright & PermissionsCopyright © 2020 by American College of Physicians. 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