AI Article Synopsis
- - Mitochondrial alterations are frequently observed in muscle biopsies of sporadic inclusion body myositis (s-IBM) and polymyositis with mitochondrial pathology, but they are less common in dermatomyositis (DM), where their impact on treatment outcomes remains unclear.
- - A case study is presented involving a 77-year-old woman with anti-Mi-2 positive dermatomyositis who exhibited severe muscle weakness and significant mitochondrial abnormalities, showing poor response to standard therapies but some improvement with anti-JAK treatment.
- - The findings suggest that mitochondrial dysfunction could indicate greater disease severity in DM, leading to worse treatment responses, and supports the exploration of JAK-inhibitors as a potential option for cases with mitochondrial abnormalities
Article Abstract
Introduction: Mitochondrial alterations are a common finding in muscle biopsy of sporadic inclusion body myositis (s-IBM) and polymyositis with mitochondrial pathology (PM-Mito). Both disorders generally have poor treatment response. Nevertheless, mitochondrial myopathology has been rarely reported in dermatomyositis (DM) outside areas of perifascicular atrophy and a relationship with therapeutic outcome is not established.
Methods: We report on clinical, immunological, radiological, and myopathological findings of a case of severe, treatment-refractory anti-Mi-2-positive DM.
Results: A 77-year-old woman developed anti-Mi-2 DM with severe diffuse muscle weakness associated with abundant mitochondrial abnormalities at muscle biopsy, beside the typical features of inflammatory myopathy. The patient was poorly responsive to multiple-line therapies and finally anti-JAK (anti-Janus activated kinase) was administered, leading to partial clinical improvement.
Discussion: Given the usual satisfactory treatment response and favorable outcome of anti-Mi-2 DM, we suppose that mitochondrial dysfunction on muscle biopsy could represent a marker of disease severity in DM, predicting a worse response to treatment and a poor clinical outcome. JAK-inhibitors could represent a good treatment option in refractory anti-Mi-2 DM with mitochondrial abnormalities.
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| http://dx.doi.org/10.1007/s10072-023-07035-w | DOI Listing |
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