A case of hyperLp(a)aemia, associated with systemic lupus erythematosus, suffering from myocardial infarction and cerebral infarction.
We describe a case of systemic lupus erythematosus (SLE) with nephrotic syndrome who suffered from myocardial infarction and cerebral infarction associated with hyperLp(a)aemia. The proband was an 18-year-old Japanese male who was found to have hypercholesterolemia and hyperLp(a)aemia, with a serum total cholesterol level of 361 mg/dl and a serum Lp(a) level of 197 mg/dl. His father and mother showed higher Lp(a)levels (26 and 56 mg/dl, respectively) than those in normals (18 +/- 0.6 mg/dl, mean +/- SE). Lp(a)glycoprotein phenotypes were examined. The proband had the phenotype S2/4, which is associated with high Lp(a) concentration. His parents had the phenotype S3/4 and S2/4. No cardiovascular diseases were noted in other members of his family. After treatment with CS-514, a competitive inhibitor of 3-hydroxy-3-methylglutaryl coenzyme A reductase. Lp(a) levels decreased from 197 to 121 mg/dl, but still remained abnormally high. LDL apheresis using a Liposorber system was attempted in this patient. Total and LDL cholesterol levels decreased by 57 and 62%, respectively. Lp(a) levels decreased by 68%. These results suggest that LDL apheresis may be an alternative therapy in drug resistant hyperLp(a)aemia.
Takegoshi
T
T
Department of Internal Medicine, Fukui Prefectural Hospital, Japan.
Haba
T
T
Hirai
J
J
Saga
T
T
Kitoh
C
C
Mabuchi
H
H
D000293:Adolescent; D000792:Angiography; D002544:Cerebral Infarction / Q000150:complications*; D017023:Coronary Angiography; D005260:Female; D006801:Humans; D006937:Hypercholesterolemia / Q000150:complications; D017270:Lipoprotein(a); D008074:Lipoproteins / Q000097:blood* / Q000378:metabolism; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D008297:Male; D009203:Myocardial Infarction / Q000150:complications*; D010375:Pedigree; D010641:Phenotype
D017270:Lipoprotein(a); D008074:Lipoproteins