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BioMed Research International
Volume 2018, Article ID 5069042, 15 pages
https://doi.org/10.1155/2018/5069042
Research Article

Clinical, Histological, and Immunohistochemical Findings in Inclusion Body Myositis

1Departamento de Neurologia, Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP, Brazil
2Disciplina de Reumatologia, Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP, Brazil
3Setor de Doenças Neuromusculares, Disciplina de Neurologia, Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brazil

Correspondence should be addressed to Edmar Zanoteli; rb.moc.arret@iletonaz

Received 29 May 2017; Revised 15 December 2017; Accepted 27 December 2017; Published 29 January 2018

Academic Editor: Vida Demarin

Copyright © 2018 Leonardo Valente de Camargo et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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, and the use of degeneration and autophagic markers can be useful for the diagnosis.