Immune-mediated necrotizing myopathy is a very rare inflammatory disease affecting skeletal muscles. Immune-mediated necrotizing myopathy may be associated with myositis-specific autoantibodies including anti-single recognition particle and anti-3-hydroxy-3- methylglutaryl-coenzyme A reductase, infectious agents (HIV or hepatitis C), other connective tissue disorders (such as scleroderma), and malignancy. We reported a 28-year-old healthy woman presented with subacute onset ascending muscle weakness 2 weeks after an annual influenza vaccination. Cerebral Spinal Fluid study showed normal cell counts with elevated protein and nerve conduction study showed reduced diffuse compound muscle action potential amplitudes suggesting a diagnosis of Guillain-Barré syndrome. Despite treatment using intravenous immunoglobulin, her condition continued to get worse with new bulbar and respiratory muscle weakness. Eventually, the diagnosis of anti-single recognition particle-mediated necrotizing myopathy was made based on elevated creatine kinase, thigh magnetic resonance imaging, muscle biopsy, and positive antibody testing. Our patient responded to the combination of intravenous immunoglobulin, prednisone, and anti-CD20 monoclonal antibody, rituximab.
Inflammatory demyelinating neuropathies have variable responses to immunomodulating therapy. Eight patients with chronic inflammatory neuropathies who were refractory to standard therapy were treated with fludarabine, a combination of fludarabine and cyclophosphamide, and in 1 case with fludarabine and rituximab. Five patients with immunoglobulin M anti-myelin-associated glycoprotein neuropathies received fludarabine. Three patients with chronic inflammatory demyelinating polyneuropathy received a combination of fludarabine and cyclophosphamide. All 8 patients improved in either functional status or strength with minimal toxicities. Most patients experienced sustained remission after the use of fludarabine or fludarabine and cyclophosphamide. Fludarabine alone or in combination with cyclophosphamide should be considered for patients with inflammatory demyelinating neuropathies, refractory to other treatments.
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