Type V hyperlipoproteinemia in systemic lupus erythematosus

Abstract

Key wordslipoprotein lipase, systemic lupus erythematosus, type V hyperlipoproteinemia.Lipoprotein lipase (LPL), which is expressed in the parenchymal cells of several extrahepatic tissues, catalyzes the hydrolysis of the triacylglycerol component of chylomicrons and very low-density lipoproteins (VLDL). Lipoprotein lipase deficiency results in type I or type V hyperlipo-proteinemia. 1 The mutation for the LPL gene is the most common cause of LPL deficiency. Rare patients with systemic lupus erythematosus (SLE) were reported to show extremely low levels of LPL activity in postheparin plasma, and to develop various types of hyperlipoproteinemia such as type I, 2-4 type IIB, 3 type III, 5 and type V. 6 The present study involves a boy with SLE complicated with type V hyperlipoproteinemia due to acquired LPL deficiency. The presenting signs were hyperlipemia and undetectable levels of LPL activity and protein mass in postheparin plasma. The patient finally developed an immunologic disorder compatible with SLE after a long period of autoimmune reaction. To our knowledge, this is the first male child case that showed a complete deficiency of LPL due to SLE.