Capillary basement membrane reduplication in myositis patients with mild clinical features of systemic sclerosis supports the concept of ‘scleromyositis’
| العنوان: | Capillary basement membrane reduplication in myositis patients with mild clinical features of systemic sclerosis supports the concept of ‘scleromyositis’ |
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| المؤلفون: | Yves Troyanov, Valérie Leclair, Benjamin Ellezam, Marie Hudson, Alain Meyer, Océane Landon-Cardinal |
| المصدر: | Acta Neuropathologica |
| بيانات النشر: | Springer Science and Business Media LLC, 2021. |
| سنة النشر: | 2021 |
| مصطلحات موضوعية: | Scleromyositis, Basement membrane, Reduplication, Original Paper, Pathology, medicine.medical_specialty, Scleroderma, Systemic, Myositis, business.industry, medicine.disease, Basement Membrane, Capillary pathology, Capillaries, Pathology and Forensic Medicine, Cellular and Molecular Neuroscience, medicine.anatomical_structure, Humans, Systemic sclerosis, Medicine, Large-scale electron microscopy, Neurology (clinical), business |
| الوصف: | Systemic sclerosis represents a chronic connective tissue disease featuring fibrosis, vasculopathy and autoimmunity, affecting skin, multiple internal organs, and skeletal muscles. The vasculopathy is considered obliterative, but its pathogenesis is still poorly understood. This may partially be due to limitations of conventional transmission electron microscopy previously being conducted only in single patients. The aim of our study was therefore to precisely characterize immune inflammatory features and capillary morphology of systemic sclerosis patients suffering from muscle weakness. In this study, we identified 18 individuals who underwent muscle biopsy because of muscle weakness and myalgia in a cohort of 367 systemic sclerosis patients. We performed detailed conventional and immunohistochemical analysis and large-scale electron microscopy by digitizing entire sections for in-depth ultrastructural analysis. Muscle biopsies of 12 of these 18 patients (67%) presented minimal features of myositis but clear capillary alteration, which we termed minimal myositis with capillary pathology (MMCP). Our study provides novel findings in systemic sclerosis-associated myositis. First, we identified a characteristic and specific morphological pattern termed MMCP in 67% of the cases, while the other 33% feature alterations characteristic of other overlap syndromes. This is also reflected by a relatively homogeneous clinical picture among MMCP patients. They have milder disease with little muscle weakness and a low prevalence of interstitial lung disease (20%) and diffuse skin involvement (10%) and no cases of either pulmonary arterial hypertension or renal crisis. Second, large-scale electron microscopy, introducing a new level of precision in ultrastructural analysis, revealed a characteristic capillary morphology with basement membrane thickening and reduplications, endothelial activation and pericyte proliferation. We provide open-access pan-and-zoom analysis to our datasets, enabling critical discussion and data mining. We clearly highlight characteristic capillary pathology in skeletal muscles of systemic sclerosis patients. Supplementary Information The online version contains supplementary material available at 10.1007/s00401-021-02305-3. |
| تدمد: | 1432-0533 0001-6322 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::e531333ef2e1ffd7aa6876b522273ec9Test https://doi.org/10.1007/s00401-021-02335-xTest |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....e531333ef2e1ffd7aa6876b522273ec9 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 14320533 00016322 |
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