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 Nov 1;33(6):522-528.
doi: 10.1097/BOR.0000000000000831.

Lymphocyte immunophenotyping in inflammatory myositis: a review

Affiliations
Review

Lymphocyte immunophenotyping in inflammatory myositis: a review

Chiara Franco et al. Curr Opin Rheumatol. .

Abstract

Purpose of review: This is a comprehensive review of the current knowledge on predominant immune cell phenotypes involved in idiopathic inflammatory myopathies (IIM).

Recent findings: Major circulating immune cell subpopulations described in IIM encompass the lymphocyte compartment. An unbalance in T cell subsets seems to consistently affect the peripheral and muscle compartment, with a predominance of CD4+ T and B cells in dermatomyositis, CD8+ T cells in polymyositis/inclusion body myositis (IBM) and novel findings highlighting novel proinflammatory T subsets, that is, CD8+Tbet+ and CD28- T cells across different IIM subsets. On the other hand, an impairment in Treg cells number and function has been described especially across polymyositis/dermatomyositis and IBM. Total T follicular helper (Tfh) cells, increased in immune-mediated necrotizing myopathy, skewed toward Tfh2 and Tfh17 in dermatomyositis, polymyositis, and juvenile dermatomyositis. B cell compartment is more rarely described in IIM, yet an unbalance in this pool is as well likely. Evidence of plasma cells increased in polymyositis, dermatomyositis, IBM, and Bregs decreased in dermatomyositis have been reported. Perturbations in the memory and naïve subsets are common in dermatomyositis/polymyositis and antisynthetase syndrome.

Summary: Protean immune cell abnormalities characterize different IIM subsets, reflecting the complexity of these autoimmune conditions. A deeper understanding of B-cell and T-cell immunophenotyping may promote early diagnosis and identification of new potential therapeutic targets.

PubMed Disclaimer

References

    1. Mariampillai K, Granger B, Amelin D, et al. Development of a new classification system for idiopathic inflammatory myopathies based on clinical manifestations and myositis-specific autoantibodies. JAMA Neurol 2018; 75:1528–1537.
    1. Schmidt J. Current classification and management of inflammatory myopathies. J Neuromuscul Dis 2018; 5:109–129.
    1. Loredo Martinez M, Zampieri S, Franco C, et al. Nonimmune mechanisms in idiopathic inflammatory myopathies. Curr Opin Rheumatol 2020; 32:515–522.
    1. Iaccarino L, Ghirardello A, Bettio S, et al. The clinical features, diagnosis and classification of dermatomyositis. J Autoimmun 2014; 48–49:122–127.
    1. Gasparotto M, Gatto M, Saccon F, et al. Pulmonary involvement in antisynthetase syndrome. Curr Opin Rheumatol 2019; 31:603–610.