Clinical, Histological, and Immunohistochemical Findings in Inclusion Body Myositis

Clinical, Histological, and Immunohistochemical Findings in Inclusion Body Myositis

Author de Camargo, Leonardo Valente Google Scholar
de Carvalho, Mary Souza Google Scholar
Shinjo, Samuel Katsuyuki Google Scholar
Bulle de Oliveira, Acary Souza Autor UNIFESP Google Scholar
Zanoteli, Edmar Google Scholar
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, alpha-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, alpha-synuclein, and TDP-43 in muscle biopsies. The sIBM has characteristic clinical and histological findings, and the use of degeneration and autophagic markers can be useful for the diagnosis.
xmlui.dri2xhtml.METS-1.0.item-coverage London
Language English
Date 2018
Published in Biomed Research International. London, v. , p. -, 2018.
ISSN 2314-6133 (Sherpa/Romeo, impact factor)
Publisher Hindawi Ltd
Extent -
Origin http://dx.doi.org/10.1155/2018/5069042
Access rights Open access Open Access
Type Article
Web of Science ID WOS:000423796100001
URI https://repositorio.unifesp.br/handle/11600/53887

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