Chylomicronemia from GPIHBP1 autoantibodies.

摘要: Some cases of chylomicronemia are caused by autoantibodies against glycosylphosphatidylinositol-anchored HDL binding protein 1 (GPIHBP1), an endothelial cell that shuttles LPL to the capillary lumen. GPIHBP1 prevent and transport GPIHBP1, thereby disrupting lipolytic processing triglyceride-rich lipoproteins. Here, we review "GPIHBP1 autoantibody syndrome" summarize clinical laboratory findings in 22 patients. All patients had chylomicronemia, but did not find a correlation between triglyceride levels levels. Many history pancreatitis, most and/or serological evidence autoimmune disease. IgA were present all patients, IgG4 19 Patients with low plasma levels, consistent impaired delivery into capillaries. Plasma measured monoclonal antibody-based ELISA, very 17 reflecting inability ELISA detect presence (immunoassay interference). However, high five indicating little capacity their interfere ELISA. Recently, several syndrome treated successfully rituximab, resulting disappearance normalization both The should be considered any patient newly acquired unexplained chylomicronemia.