Therapeutic Uncertainty in Statin-Induced Necrotizing Autoimmune Myopathy: A Case Highlighting Limited Recovery Despite Aggressive Treatment
Maria Chacon1, Linker Viñan Paucar2, Daniel Moreno-Zambrano3, Rocio Santibanez4, Carlos Noboa-Bejarano5
1Universidad de Especialidades Espíritu Santo, 2Universidad Católica Santiago de Guayaquil, 3Department of Neurology, Larkin Community Hospital, South Miami Campus, 4Universidad Catolica de Santiago de Guayaquil, 5Grupo Hospitalario Kennedy, Guayaquil, Ecuador
Objective:
To present a case of Statin-induced necrotizing autoimmune myopathy (SINAM) in the absence of standardized guidelines.
Background:

Statins are widely prescribed and generally well tolerated for cholesterol reduction, yet in rare cases they can trigger immune-mediated complications. SINAM manifests with progressive proximal muscle weakness, elevated creatine kinase, and anti-HMG-CoA reductase antibodies. Its incidence is estimated at 2–3 cases per 100,000 exposed individuals. Prompt recognition is essential, as early initiation of high-dose glucocorticoids and additional immunosuppressive agents can improve outcomes. Notably, SINAM can also affect children and statin-naïve adults, reflecting its heterogeneous triggers and complex pathophysiology.


Design/Methods:
NA
Results:

A 73-year-old male presented with a one-year history of progressive flaccid paraparesis, dysphagia, and loss of independent ambulation. His medical history was notable for chronic statin therapy at 80 mg daily over the past four years and coronary stent placement for aortic stenosis. Neurological examination revealed symmetric lower-limb weakness without sensory or sphincter involvement, absent reflexes, and no upper motor neuron signs.

Electromyography showed severe inflammatory myopathy with denervation potentials and rapid recruitment of small motor units. Quadriceps ultrasound revealed structural abnormalities. Laboratory evaluation revealed CK 11,600 U/L and myoglobin 3,100 ng/mL, with positive anti-HMG-CoA reductase antibodies, confirming SINAM.

Initial treatment with intravenous fluids and IVIG (400 mg/kg/day × 5 days) resulted in significant improvement (CK 3,117 U/L, myoglobin 400 ng/mL). However, relapse occurred with recurrent weakness, and CK 5,617 U/L. High-dose corticosteroids pulses were ineffective. A second IVIG course combined with azathioprine titrated to 150 mg/day achieved approximately 60% functional recovery.

Conclusions:
SINAM remains a challenge due to the lack of standardized treatment guidelines and the highly variable response to treatment. Although IVIG can lead to marked initial improvement, sustained recovery may be difficult despite corticosteroids and additional immunosuppression. Further research and consensus protocols are needed to improve treatment strategies and long-term outcomes.
10.1212/WNL.0000000000212859
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