Thrombospondin-1 mediates muscle damage in brachio-cervical inflammatory myopathy and systemic sclerosis.

Neurology. Neuroimmunology and Neuroinflammation
Xavier Suárez-CalvetJordi Díaz-Manera

Abstract

To describe the clinical, serologic and histologic features of a cohort of patients with brachio-cervical inflammatory myopathy (BCIM) associated with systemic sclerosis (SSc) and unravel disease-specific pathophysiologic mechanisms occurring in these patients. We reviewed clinical, immunologic, muscle MRI, nailfold videocapillaroscopy, muscle biopsy, and response to treatment data from 8 patients with BCIM-SSc. We compared cytokine profiles between patients with BCIM-SSc and SSc without muscle involvement and controls. We analyzed the effect of the deregulated cytokines in vitro (fibroblasts, endothelial cells, and muscle cells) and in vivo. All patients with BCIM-SSc presented with muscle weakness involving cervical and proximal muscles of the upper limbs plus Raynaud syndrome, telangiectasia and/or sclerodactilia, hypotonia of the esophagus, and interstitial lung disease. Immunosuppressive treatment stopped the progression of the disease. Muscle biopsy showed pathologic changes including the presence of necrotic fibers, fibrosis, and reduced capillary number and size. Cytokines involved in inflammation, angiogenesis, and fibrosis were deregulated. Thrombospondin-1 (TSP-1), which participates in all these 3 processes, was upr...Continue Reading

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Citations

Aug 17, 2020·Current Opinion in Neurology·Claudia Nuñez-PeraltaJordi Díaz-Manera
Dec 31, 2020·BioMed Research International·D JiangK Tao
Mar 16, 2021·Matrix Biology Plus·Silvia Redondo-GarcíaJuan Carlos Rodríguez-Manzaneque
May 21, 2021·Neurology. Neuroimmunology and Neuroinflammation·Matteo LucchiniGiorgio Tasca

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Methods Mentioned

BETA
biopsies
genotyping
Profiler
ELISA
biopsy

Software Mentioned

ImageJ
Macro Angiogenesis Analyzer
Image Studio
GraphPad
NIH

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