Clinical, Histological, and Immunohistochemical Findings in Inclusion Body Myositis

BioMed Research International
Leonardo Valente de CamargoEdmar Zanoteli

Abstract

Sporadic inclusion body myositis (sIBM) is considered the most common acquired myopathy aged over 50 years. The disease is characterized by a particular process of muscle degeneration characterized by abnormal deposit of protein aggregates in association with inflammation. The aim of this study was to present clinical and muscle histopathological findings, including immunostaining for LC3B, p62, α-synuclein, and TDP-43, in 18 patients with sIBM. The disease predominated in males (61%) and European descendants, with onset of clinical manifestations around 59 years old. The most common symptoms were muscle weakness, falls, dysphagia, and weight loss. Hypertension was the main comorbidity. Most of the cases presented with paresis predominantly proximal in lower limbs and distal in upper limbs. Immunosuppressive treatment showed to be not effective. Muscle histological findings included dystrophic changes, endomysial inflammation, increased lysosomal activity, and presence of rimmed vacuoles and of beta-amyloid accumulation, in addition to high frequency of mitochondrial changes. There was increased expression of LC3B, p62, α-synuclein, and TDP-43 in muscle biopsies. The sIBM has characteristic clinical and histological findings, a...Continue Reading

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Citations

Feb 15, 2019·Current Rheumatology Reports·Júlio Brandão GuimarãesArtur R C Fernandes
Apr 12, 2019·Muscle & Nerve·Samah K AburahmaDianna Quan
Sep 4, 2019·Aging Cell·Chang-Yi CuiLuigi Ferrucci
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Jul 30, 2019·Annals of Clinical and Translational Neurology·André M S SilvaEdmar Zanoteli
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Jan 13, 2021·Muscle & Nerve·Marie-Hélène LelièvreBruno-Pierre Dubé
May 30, 2021·Ultrasound in Medicine & Biology·Julio Brandao GuimaraesEdmar Zanoteli

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Methods Mentioned

BETA
biopsies
Biopsy
light microscopy
hysterectomy

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