Fettstoffwechselstörungen und Statinunverträglichkeit bei Myotonen Dystrophien

Lipid metabolism alteration and statin intake in myotonic
dystrophies A. Schmidt, MS a; L. Kirzinger, MS a, W. Kress, PhD b,
and B. Schoser, MD a a Friedrich-Baur Institute, Department of
Neurology, Ludwig Maximilians University Munich, Munich, Germany b
Institute of Human Genetics, University of Würzburg, Würzburg,
Germany. Background: There have been no systematic reports on lipid
metabolism alteration in myotonic dystrophies. We assessed the
frequency, type, and severity of lipid alteration in both types of
DM. Furthermore, the frequency of statin intake and side effects
was investigated. Methods: A retrospective multicenter study was
conducted. 134 DM2 patients and 29 DM1 patients participated by
completing questionnaires. Additionally, their clinical and
serological records, including blood lipid parameters for
dyslipoproteinemia (cholesterol, HDL, LDL and TG) were screened and
reviewed. Results: 71.6% of DM2 and 55.2% of DM1 patients show
evidence to suffer from dyslipoproteinemia. In DM2 and DM1 patients
the age of onset of dyslipoproteinemia was 47.6y±15.1 and
46.2y±9.7, respectively. Comparing both DM groups, all measured
parameters did not differ statistically significant. Nevertheless,
comparing all lipid parameters of both types of DM with an average
German population data set, we could find a no significant
discrepancy for DM1 patients (Cholesterol: p=0.435; HDL: p=0.129),
but for DM2 patients (Cholesterol p=0.021; HDL: p=0.001). 23.1% of
DM2 and 11.1% of DM1 patients were treated with statins. 50% of all
patients discontinued statin therapy because of side effects.
Conclusions: According to Robert-Koch-Institute, Germany, one third
of German adults show elevated cholesterol levels, which is
significantly lower than the rate found here in DM patients. With
this high rate of dyslipoproteinemia in DMs, it seems to be
obvious, that DM patients show a predisposition for this metabolic
disorder. Furthermore, compared with data from
Robert-Koch-Institute, DM patients (DM2 23.1%, DM1 11.1%) use
significantly more statins than the German average. In addition,
the rate of discontinuation is high based on side effects like
augmented symptoms (hyperCKemia in 27.8% DM2, muscle pain or
weakness in 33.3% DM2) in relation to former studies revealing
statin side effects in one of 10000 patients.