Pathological findings with vacuoles in anti-mitochondrial antibody-positive inflammatory myopathy
Abstract 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 Pek...
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| Format: | Article |
| Language: | English |
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BMC
2024-04-01
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| Series: | BMC Musculoskeletal Disorders |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s12891-023-06941-6 |
| _version_ | 1797220084985364480 |
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| author | Yuanchong Chen Wei Zhang He Lv Zhaoxia Wang Hongjun Hao Yun Yuan Yiming Zheng |
| author_facet | Yuanchong Chen Wei Zhang He Lv Zhaoxia Wang Hongjun Hao Yun Yuan Yiming Zheng |
| author_sort | Yuanchong Chen |
| collection | DOAJ |
| description | Abstract 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. Their clinical records were retrospectively reviewed and the data was extracted. All the 3 cases underwent muscle biopsy. Results Three middle-aged patients presented with chronic-onset weakness of proximal limbs, marked elevation of creatine kinase, and AMA-positivity. Two of the 3 cases meet the criteria of primary biliary cholangitis. All the 3 cases presented with cardiac involvement and proteinuria. Two cases developed type 2 respiratory failure. MRI of the thigh muscle showed multiple patches of edema bilaterally in both cases, mostly in the adductor magnus. Pathological findings include degeneration of muscle fibers, diffused MHC-I positivity, and complement deposits on cell membranes. Vacuoles without rims of different sizes were discovered under the membrane of the muscle fibers. A few RBFs were discovered in case 1, while a diffused proliferation of endomysium and perimysium was shown in case 2. Conclusions AMA-positive inflammatory myopathy is a disease that could affect multiple systems. Apart from inflammatory changes, the pathological findings of muscle can also present vacuoles. |
| first_indexed | 2024-04-24T12:43:55Z |
| format | Article |
| id | doaj.art-b1c684394d2446afa249fb8a3eaa3d53 |
| institution | Directory Open Access Journal |
| issn | 1471-2474 |
| language | English |
| last_indexed | 2024-04-24T12:43:55Z |
| publishDate | 2024-04-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Musculoskeletal Disorders |
| spelling | doaj.art-b1c684394d2446afa249fb8a3eaa3d532024-04-07T11:05:40ZengBMCBMC Musculoskeletal Disorders1471-24742024-04-012511710.1186/s12891-023-06941-6Pathological findings with vacuoles in anti-mitochondrial antibody-positive inflammatory myopathyYuanchong Chen0Wei Zhang1He Lv2Zhaoxia Wang3Hongjun Hao4Yun Yuan5Yiming Zheng6Department of Neurology, Peking University First HospitalDepartment of Neurology, Peking University First HospitalDepartment of Neurology, Peking University First HospitalDepartment of Neurology, Peking University First HospitalDepartment of Neurology, Peking University First HospitalDepartment of Neurology, Peking University First HospitalDepartment of Neurology, Peking University First HospitalAbstract 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. Their clinical records were retrospectively reviewed and the data was extracted. All the 3 cases underwent muscle biopsy. Results Three middle-aged patients presented with chronic-onset weakness of proximal limbs, marked elevation of creatine kinase, and AMA-positivity. Two of the 3 cases meet the criteria of primary biliary cholangitis. All the 3 cases presented with cardiac involvement and proteinuria. Two cases developed type 2 respiratory failure. MRI of the thigh muscle showed multiple patches of edema bilaterally in both cases, mostly in the adductor magnus. Pathological findings include degeneration of muscle fibers, diffused MHC-I positivity, and complement deposits on cell membranes. Vacuoles without rims of different sizes were discovered under the membrane of the muscle fibers. A few RBFs were discovered in case 1, while a diffused proliferation of endomysium and perimysium was shown in case 2. Conclusions AMA-positive inflammatory myopathy is a disease that could affect multiple systems. Apart from inflammatory changes, the pathological findings of muscle can also present vacuoles.https://doi.org/10.1186/s12891-023-06941-6Anti-mitochondrial antibodyInflammatory myopathyMuscle MRIMuscle pathology |
| spellingShingle | Yuanchong Chen Wei Zhang He Lv Zhaoxia Wang Hongjun Hao Yun Yuan Yiming Zheng Pathological findings with vacuoles in anti-mitochondrial antibody-positive inflammatory myopathy BMC Musculoskeletal Disorders Anti-mitochondrial antibody Inflammatory myopathy Muscle MRI Muscle pathology |
| title | Pathological findings with vacuoles in anti-mitochondrial antibody-positive inflammatory myopathy |
| title_full | Pathological findings with vacuoles in anti-mitochondrial antibody-positive inflammatory myopathy |
| title_fullStr | Pathological findings with vacuoles in anti-mitochondrial antibody-positive inflammatory myopathy |
| title_full_unstemmed | Pathological findings with vacuoles in anti-mitochondrial antibody-positive inflammatory myopathy |
| title_short | Pathological findings with vacuoles in anti-mitochondrial antibody-positive inflammatory myopathy |
| title_sort | pathological findings with vacuoles in anti mitochondrial antibody positive inflammatory myopathy |
| topic | Anti-mitochondrial antibody Inflammatory myopathy Muscle MRI Muscle pathology |
| url | https://doi.org/10.1186/s12891-023-06941-6 |
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