Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2021 Jul 1;23(7):56.
doi: 10.1007/s11926-021-01017-7.

Updates on the Immunopathology in Idiopathic Inflammatory Myopathies

Akinori Uruha  1   2 , Hans-Hilmar Goebel  3   4 , Werner Stenzel  3   5
Affiliations
Review

Updates on the Immunopathology in Idiopathic Inflammatory Myopathies

Akinori Uruha et al. Curr Rheumatol Rep. .

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. 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.

Keywords: Immunohistochemistry; KLRG1; Membrane attack complex; Muscle pathology; MxA; p62.

PubMed Disclaimer

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
    1. Uruha A, Nishikawa A, Tsuburaya RS, Hamanaka K, Kuwana M, Watanabe Y, et al. Sarcoplasmic MxA expression: a valuable marker of dermatomyositis. Neurology. 2017;88(5):493–500. - PubMed
    1. Greenberg SA, Pinkus JL, Pinkus GS, Burleson T, Sanoudou D, Tawil R, et al. Interferon-alpha/beta-mediated innate immune mechanisms in dermatomyositis. Ann Neurol. 2005;57(5):664–78. - PubMed
    1. Salajegheh M, Kong SW, Pinkus JL, Walsh RJ, Liao A, Nazareno R, et al. Interferon-stimulated gene 15 (ISG15) conjugates proteins in dermatomyositis muscle with perifascicular atrophy. Ann Neurol. 2010;67(1):53–63. - PubMed - PMC
    1. Suárez-Calvet X, Gallardo E, Nogales-Gadea G, Querol L, Navas M, Díaz-Manera J, et al. Altered RIG-I/DDX58-mediated innate immunity in dermatomyositis. J Pathol. 2014;233(3):258–68. - PubMed
    1. De Luna N, Suárez-Calvet X, Lleixà C, Diaz-Manera J, Olivé M, Illa I, et al. Hypoxia triggers IFN-I production in muscle: implications in dermatomyositis. Sci Rep. 2017;7(1):8595. - PubMed - PMC

LinkOut - more resources