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Anti-mitochondrial M2 antibody (AMA-M2)-positive myositis is an idiopathic inflammatory myopathy (IIM). Of all patients with myositis, 2.5-19.5% have AMA-M2 antibodies. However, the detailed distribution of muscles affected in AMA-positive myositis is unknown. Therefore, we examined lower muscle magnetic resonance imaging (MRI) findings of patients with AMA-positive myositis. Among the 63 patients with IIM at our institute, 5 (7.9%) were positive for AMA-M2 antibodies. However, one was also positive for anti-Jo1 antibodies; therefore, four patients were finally participated in this study. All patients had high-intensity MRI signals in the proximal muscles, including the gluteus maximus and iliopsoas muscles, and in the thigh muscles, including the vastus lateralis, vastus medialis, adductor magnus, and semimembranosus muscles. Lower leg muscles were relatively spared. Fascial edema was observed in all patients and was also present in the lower leg muscles. Subcutaneous edema was observed, particularly in the proximal portion of the lower limbs. In AMA-positive myositis, proximal muscles, including the gluteus maximus, vastus lateralis, adductor magnus, and the semimembranosus, were markedly affected, while the lower leg muscles were relatively preserved. Additionally, fascial edema was evident even in lower leg muscles. Therefore, muscle MRI can be a useful diagnostic aid for AMA-positive myositis.
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http://dx.doi.org/10.1016/j.nmd.2023.07.005 | DOI Listing |
Clin Rheumatol
June 2025
Department of Rheumatology, Tohoku University Hospital, Sendai, Miyagi, Japan.
Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of autoimmune diseases characterized primarily by muscle inflammation. Anti-mitochondrial antibodies (AMA) are typically associated with primary biliary cholangitis; however, they have also been linked to a rare subgroup of IIMs, namely "AMA-positive myositis," marked by a chronic disease course and muscle weakness, particularly affecting the paravertebral muscles, alongside muscle atrophy and cardiopulmonary complications. We report the case of a 40-year-old woman diagnosed with AMA-positive myositis, predominantly involving axial myopathy complicated by multiple arrhythmias and biopsy-proven focal segmental glomerulosclerosis with mitochondrial abnormalities on electron microscopy.
View Article and Find Full Text PDFIntern Med
March 2025
Departments of Cardiovascular Medicine, Tohoku University Graduate School of Medicine, Japan.
A 69-year-old woman presented with heart failure and progressive muscle weakness and was diagnosed as anti-mitochondrial antibody (AMA) myositis with cardiac involvement. Immunosuppressive therapy with prednisolone and intravenous cyclophosphamide significantly improved the symptoms, hemodynamics, and cardiac function. Cardiac magnetic resonance (CMR) T1 and T2 mapping showed elevated native T1, T2, and extracellular volume fractions during heart failure exacerbation (day 37) compared to pre-hospitalization values (10 months before admission) and follow-up conducted 6 and 12 months after admission.
View Article and Find Full Text PDFMedicine (Baltimore)
December 2024
Department of Neurology, Showa University Fujigaoka Hospital, Yokohama, Kanagawa, Japan.
Rationale: Anti-mitochondrial antibodies (AMA) M2-positive myositis can lead to severe respiratory failure. Traditional immunotherapies sometimes fail to address respiratory failure. Herein, this CARE-compliant case report described a patient with AMA-M2-positive myositis who recovered from ventilation with tracheostomy owing to immunotherapy-resistant respiratory failure to spontaneous breathing after modified lung volume recruitment (mLVR) therapy.
View Article and Find Full Text PDFJ Neurol Sci
December 2024
Department of Neurology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan. Electronic address:
BMC Musculoskelet Disord
April 2024
Department of Neurology, Peking University First Hospital, No.8 Xishiku Street, Xicheng District, Beijing, 100034, China.
Background: A few patients with inflammatory myopathy showed anti-mitochondrial antibody (AMA) positivity. This study aimed to report the clinical and pathological findings with vacuoles in 3 cases of such patients.
Methods: Three cases with myositis from the Myositis Clinical Database of Peking University First Hospital were identified with AMA positivity.