Erschienen in:
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
Einloggen, um Zugang zu erhalten
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.