Prominent mitochondrial pathology in a case of refractory dermatomyositis: coincidence or concause?

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 i...

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Published in:Neurological sciences 2023-12, Vol.44 (12), p.4525-4529
Main Authors: Lauletta, Antonio, De Carolis, Lanfranco, Teresi, Valentina, Fionda, Laura, Leonardi, Luca, Ceccanti, Marco, Costanzo, Rocco, Rossini, Elena, Tufano, Laura, Merlonghi, Gioia, Morino, Stefania, Garibaldi, Matteo
Format: Article
Language:English
Subjects:
Atrophy
Biopsy
Brief Communication
Dermatomyositis
Inflammation
Medicine
Medicine & Public Health
Mitochondria
Myopathy
Myositis
Neurology
Neuroradiology
Neurosciences
Neurosurgery
Pathology
Polymyositis
Psychiatry
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Summary: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.
ISSN:1590-1874
1590-3478
DOI:10.1007/s10072-023-07035-w