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Erschienen in: Current Rheumatology Reports 7/2021

01.07.2021 | Inflammatory Muscle Disease (L Diederichsen and H Chinoy, Section Editor)

Updates on the Immunopathology in Idiopathic Inflammatory Myopathies

verfasst von: Akinori Uruha, Hans-Hilmar Goebel, Werner Stenzel

Erschienen in: Current Rheumatology Reports | Ausgabe 7/2021

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Abstract

Purpose of Review

To review recent advances in immunopathology for idiopathic inflammatory myopathies, focusing on widely available immunohistochemical analyses.

Recent Findings

Sarcoplasmic expression of myxovirus resistance protein A (MxA) is specifically observed in all types of dermatomyositis and informs that type I interferons are crucially involved in its pathogenesis. It is a more sensitive diagnostic marker than perifascicular atrophy. Diffuse tiny dots in the sarcoplasm highlighted by p62 immunostaining are characteristically seen in immune-mediated necrotizing myopathy. This feature is linked to a chaperone-assisted selective autophagy pathway. Myofiber invasion by highly differentiated T cells, a marker of which is KLRG1, is specific to inclusion body myositis and has a crucial role in its pathogenesis.

Summary

The recent advances in immunopathology contribute to increased diagnostic accuracy and a better understanding of the underlying pathophysiology in different types of idiopathic inflammatory myopathies.
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Metadaten
Titel
Updates on the Immunopathology in Idiopathic Inflammatory Myopathies
verfasst von
Akinori Uruha
Hans-Hilmar Goebel
Werner Stenzel
Publikationsdatum
01.07.2021
Verlag
Springer US
Erschienen in
Current Rheumatology Reports / Ausgabe 7/2021
Print ISSN: 1523-3774
Elektronische ISSN: 1534-6307
DOI
https://doi.org/10.1007/s11926-021-01017-7

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