Characterization of a New Case of Autoimmune Type I Hyperlipidemia: Long-Term Remission under Immunosuppressive Therapy1
Valerie Pruneta,
Philippe Moulin2,
Florence Labrousse,
Pierre-Jean Bondon,
Gabriel Ponsin and
Francois Berthezene
Laboratoire de Métabolisme des Lipides (V.P., P.M., G.P.),
Service d’Endocrinologie et des Maladies de la Nutrition (F.L., F.B.),
and Laboratoire de Biochimie (P.B.), Hôpital de l’Antiquaille,
Lyon, France
Address all correspondence and requests for reprints to: Dr. Valérie Pruneta, Laboratoire de Métabolisme des Lipides, Hôpital de l’Antiquaille, 1 rue de l’Antiquaille, 69005 Lyon, France.
Only a few cases of type I hyperlipidemia occurring in patientswith
autoimmune disease have been reported. We describe thecase of a
35-yr-old woman suffering from severe type I hyperchylomicronemia.A
combination of various hypolipidemic treatments, includingstrict
hypolipidemic dietary therapy and administration of fibratesor n-3
fatty acids, was inefficient. Because of a history offamilial
autoimmunity, we introduced an immunosuppressive therapythat resulted
in consistent long term and stable remission.Two attempts to reduce
the immunosuppressor dose resulted inmajor relapses. To explain the
defect of chylomicron hydrolysis,we investigated the postheparin
plasma lipase activities. Hepatictriglyceride lipase activity was
normal, whereas that of lipoproteinlipase (LPL) was reduced to about
30% of normal. Immunosuppressivetherapy resulted in a complete and
durable normalization ofLPL activity. Using Western blot analysis, we
found in the plasmaof the patient a circulating IgG specifically
directed againstLPL, which became undetectable during
immunosuppressive therapy.Western blot analysis revealed that the
whole circulating anti-LPLautoantibody was bound to chylomicrons.
Proteins extracted frompatient’s chylomicrons were able to induce a
dose-relatedinhibition of LPL activity in vitro,
whereas that of hepatictriglyceride lipase remained unchanged.
These data constitute the first description of autoimmune
hyperchylomicronemiadue to an exclusive defect of LPL activity, and
they show thata complete remission has been obtained after
immunosuppressivetherapy. Finally, our finding that the anti-LPL
autoantibodyis bound to chylomicrons emphasizes their previously
unrecognizedability to transport LPL, already described for other
lipoproteinfractions.
This article has been cited by other articles:
M. Michel, E. Fois, M. Niault, V. Pruneta, B. Godeau, and M. Michel Severe autoimmune type V hyperlipidemia preceding a systemic lupus erythematosus in a 15 year-old girl
Lupus,
May 1, 2007;
16(5):
378 - 379.
[PDF]
V. Pruneta-Deloche, C. Marcais, L. Perrot, A. Sassolas, M. Delay, B. Estour, M. Lagarde, and P. Moulin Combination of Circulating Antilipoprotein Lipase (Anti-LPL) Antibody and Heterozygous S172 fsX179 Mutation of LPL Gene Leading to Chronic Hyperchylomicronemia
J. Clin. Endocrinol. Metab.,
July 1, 2005;
90(7):
3995 - 3998.
[Abstract][Full Text][PDF]
V. Pruneta, D. Autran, G. Ponsin, C. Marcais, L. Duvillard, B. Verges, F. Berthezene, and P. Moulin Ex Vivo Measurement of Lipoprotein Lipase-Dependent Very Low Density Lipoprotein (VLDL)-Triglyceride Hydrolysis in Human VLDL: An Alternative to the Postheparin Assay of Lipoprotein Lipase Activity?
J. Clin. Endocrinol. Metab.,
February 1, 2001;
86(2):
797 - 803.
[Abstract][Full Text]
V. Pruneta, T. Pulcini, F. Lalanne, C. Marcais, F. Berthezene, G. Ponsin, and P. Moulin VLDL-bound lipoprotein lipase facilitates the cholesteryl ester transfer protein-mediated transfer of cholesteryl esters from HDL to VLDL
J. Lipid Res.,
December 1, 1999;
40(12):
2333 - 2339.
[Abstract][Full Text][PDF]
