Myositis associated with antimitochondrial antibodies (AMAs) is characterized by proximal and axial muscle weakness and cardiac involvement.1-4 The muscle pathology most commonly manifests as necrotizing myopathy with a variable extent of inflammatory cell infiltrates. Granulomatous inflammation has been observed in 25% of myositis patients.1 However, the underlying pathomechanism remains poorly understood. Here we describe a patient with myositis associated with AMA characterized by the presence of rimmed vacuoles (RV) in the biopsied muscle, suggesting the involvement of a degenerative process.
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