Abstract
BackgroundStatin-associated necrotizing myopathy (SANM) is a rare autoimmune disorder caused by administration of statins. SANM is characterized by weakness due to necrosis and regeneration of myofibers. Here we report the first case of SANM with acute respiratory failure treated with noninvasive pressure support ventilation in addition to immunosuppressants.Case presentationA 59-year-old woman who had been treated with 2.5 mg/day of rosuvastatin calcium for 5 years stopped taking the drug 4 months before admission to our hospital due to elevation of creatine kinase (CK). Withdrawal of rosuvastatin for 1 month did not decrease the level of CK, and she was admitted to our hospital due to the development of muscle weakness of her neck and bilateral upper extremities. Anti-3-hydroxy-3-methylglutaryl coenzyme A reductase antibodies were positive. Magnetic resonance imaging showed myositis, and muscle biopsy from the right biceps brachii muscle showed muscle fiber necrosis and regeneration without inflammatory cell infiltration, suggesting SANM. After the diagnosis, she received methylprednisolone pulse therapy (mPSL, 1 g/day × 3 days, twice) and subsequent oral prednisolone therapy (PSL, 30 mg/day for 1 month, 25 mg/day for 1 month and 22.5 mg/day for 1 month), leading to improvement of her muscle weakness. One month after the PSL tapering to 20 mg/day, her muscle weakness deteriorated with oxygen desaturation (SpO2: 93% at room air) due to hypoventilation caused by weakness of respiratory muscles. BIPAP was used for the management of acute respiratory failure in combination with IVIG (20 g/day × 5 days) followed by mPSL pulse therapy (1 g/day × 3 days), oral PSL (30 mg/day × 3 weeks, then tapered to 25 mg/day) and tacrolimus (3 mg/day). Twenty-seven days after the start of BIPAP, she was weaned from BIPAP with improvement of muscle weakness, hypoxemia and hypercapnia. After she achieved remission with improvement of muscle weakness and reduction of serum CK level to a normal level, the dose of oral prednisolone was gradually tapered to 12.5 mg/day without relapse for 3 months.ConclusionsOur report provides new insights into the role of immunosuppressants and biphasic positive airway pressure for induction of remission in patients with SANM.
Highlights
Statin-associated necrotizing myopathy (SANM) is a rare autoimmune disorder caused by administration of statins
Statin-associated necrotizing myopathy (SANM), characterized by symmetrical weakness and muscle enzyme elevations as a consequence of necrosis and regeneration of myofibers, is a rare disease that occurs in patients treated with statins [1,2,3]
Noninvasive pressure support ventilation (NIPSV) is the delivery of mechanical ventilation to the lungs without an endotracheal airway by biphasic positive airway pressure (BIPAP) that is applied with two levels of pressure, inspiratory positive airway pressure (IPAP) and expiratory positive airway pressure (EPAP) [10]
Summary
Statin-associated necrotizing myopathy (SANM), characterized by symmetrical weakness and muscle enzyme elevations as a consequence of necrosis and regeneration of myofibers, is a rare disease that occurs in patients treated with statins [1,2,3]. The patient’s inspiratory effort triggers the ventilator to deliver a decelerated flow to achieve and maintain a preset pressure, while ventilatory assistance ceases when the patient’s inspiratory flow falls This modality is useful for patients with hypoxemia and/or hypercapnia due to acute respiratory failure, leading to a reduction of the intubation rate and mortality. CRP C-reactive protein, RF rheumatoid factor, ANA anti-nuclear antibodies, Jo-1 anti-Jo-1 antibodies, ARS anti-aminoacyl-tRNA synthetase antibodies, HMGCR anti-3hydroxy-3-methylglutaryl coenzyme A reductase antibodies level (126 U/L) to a normal level (Fig. 1f and g) After she achieved remission, the dose of oral prednisolone was gradually tapered to 12.5 mg /day without relapse for 3 months
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