Abstract
Statins have become the commonest lipid-lowering agent worldwide and have significantly reduced morbidity and mortality associated with cardiovascular diseases. Overall, statins are very well tolerated. However, in clinical practice, a wide variety of skeletal myopathic effects have been observed, ranging from asymptomatic patients with high creatine phosphokinase (CPK) to fatal cases of acute rhabdomyolysis. Recent reports suggest that statins are associated with immune-mediated necrotizing myopathy (IMNM), a unique autoimmune myopathy. Unlike other drug reactions, this can occur months to years after initiation of statin. It is a distinctive autoimmune myopathy where symptoms persist or even progress after statin discontinuation and requires immunosuppressive therapy. The presence of anti-hydroxy-methyl-glutaryl coenzyme-A reductase (HMGCR) antibody in serum strengthens the diagnosis of statin-associated necrotizing myopathy. Here we present a case of statin-associated IMNM in a 43-year-old Caucasian female who had statin-induced progressive deterioration of proximal muscle weakness with poor response to high-dose steroids and required further immunosuppressive therapy.
Highlights
Statins, called hydroxy-methyl-glutaryl-coenzyme-A reductase (HMGCR) inhibitors, are generally considered safe and are well-tolerated drugs
A novel entity of statin-associated immune-mediated necrotizing myopathy (IMNM) has been reported, which is distinct from statin-induced myalgias and inflammatory myopathies mentioned above
Specific autoantibody directed against hydroxy-methyl-glutaryl coenzyme-A reductase (HMGCR) in patients with IMNM can help identify these patients in clinical practice and determine the need for immunosuppressive therapy
Summary
Called hydroxy-methyl-glutaryl-coenzyme-A reductase (HMGCR) inhibitors, are generally considered safe and are well-tolerated drugs. The estimated incidence of IMNM is approximately two to three of every 100,000 patients treated with statins [7] These autoimmune myopathies are characterized clinically by symmetric proximal muscle weakness, elevated serum CPK levels, and myopathic findings on electromyography (EMG) [8]. Specific autoantibody directed against HMGCR in patients with IMNM can help identify these patients in clinical practice and determine the need for immunosuppressive therapy This autoimmune necrotizing myopathy, where symptoms persist or even progress after statin discontinuation, responds well after initiation of immunosuppressive therapy [5]. Clinical symptoms of significant muscle weakness, very high CPK level, and abnormal MRI findings initially raised suspicion for inflammatory myositis. In outpatient follow-up, the patient reported resolution of all muscle symptoms, CPK had been normalized and the patient did not require any further immunosuppressive drugs
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