Algorithm of management of patients with suspected statininduced myopathy (clinical case of severe congenital myopathy developed along with of long-term statin therapy)

The purpose of clinical supervision is to demonstrate a case of severe congenital myopathy and liver injury for patient of older age, that took statins (simvastatin) for a long time; to discuss the algorithm of management of patients with suspected pathology of the muscular system (rhabdomyolysis); in order to draw the attention of physicians to the need for a thorough and detailed examination of the patients with persistent high numbers creatinephosphokinase (CPK) and transaminase blood after discontinuation of statins to exclude more rare diseases - congenital myopathies. This clinical case demonstrates the development of hereditary myopathy, which for a long time, masked itself as statininduced myopathy pathology.Peculiarities of the clinical observations. The patient with high numbers of cholesterol (LDL) had been prescribed statins. During 4 years (since 2006), she regularly took these drugs and controlled biochemical parameters of blood, which remained within normal values, the cholesterol level was normalized. For the last year before admission to the Clinic of internal diseases named after Vasilenko for survey about different diseases, she has received many drugs that enhance hepatotoxic and myotoxicity action of statins. Since 2010, the patient noted the emergence of gradually increasing neurological symptoms. 6-7-fold increase in transaminases was marked in the blood during this period. For a long time, the patient was not studied CPK level, when myopathy was suspected first time, the CPK level was 13 upper limits of normal. But even after discontinuation of statins the CPK level had not normalized, but had continued steadily to increase. Differential diagnosis was conducted and possible diseases with increased levels of transaminases and CPK were excluded. According to the algorithm for management of patients with suspected statininduced defeat muscular system, the patient had been conducted electroneuromyography, liver biopsy and skeletal muscle, resulting in the unlikelihoodof statininduced myopathy as a diagnosis. Positron emission tomography and studied the tumor markers were conducted for patient to exclude a paraneoplastic process. Data for cancer process hadnot been received. Finally, the patient had been sent to the Research center of neurology RAMS, where the following diagnosis had been formulated after a survey: "oculopharyngeal muscular dystrophy" (hereditary-degenerative disease characterized by progressive muscular weakness and atrophy).Conclusion. As of today, statins are an integral component of primary and secondary prevention and treatment of cardiovascular diseases and their complications. This class of drugs confirmed and proved its effectiveness and safety by multicenter studies. However, making a decision about the need for statin use, the nature of their regime and dose, it is necessary to consider their interactions with other prescribed drugs, characteristics of the patient, which can increase the risk of statin induced myopathy. In addition, assessing the potential risk of adverse effects of statins, preference must be given to hydrophilic statins with the lowest risk of toxic effects on hepatobiliary and muscular system.

statins, myopathy, rhabdomyolysis, creatinephosphokinase, hereditary myopathy, oculopharyngeal muscular dystrophy