Peripheral blood lymphocyte subset repertoires are biased and reflect clinical features in patients with dermatomyositis

Abstract

Objective: Dermatomyositis (DM) is an idiopathic inflammatory myopathy which often involves the lungs. DM is likely to be associated with aberrant T- and B-cell activation in the pathogenesis because of the proven effectiveness of T- and B-cell-targeted treatments. Assuming that the aberrant activation is reflected by biases in the lymphocyte subset repertoires, we aimed to elucidate these biases, especially in relation to clinical features of DM.

Method: Based on the immunophenotyping standardized by the Human Immunology Project Consortium, untreated 13 DM patients, including seven patients with interstitial lung disease (ILD), and 18 age-matched healthy donors (HDs) were examined for proportions of peripheral blood lymphocyte subsets. Six DM patients were examined before and after successful induction of remission.

Results: Naïve CD4⁺ T cells and naïve B cells were more abundant, while there were fewer naïve CD8⁺ T cells, central memory CD8⁺ T cells, effector memory CD4⁺ T cells, Th1 cells, Tfh cells, and memory B cells in DM patients than in HDs. When the patients were subgrouped according to the presence of ILD, the lymphocyte subset repertoires in the patients with ILD contributed to the statistical differences in all the biased lymphocyte subset proportions. After treatment, transitional B cells vanished and there was an increase in memory B cells.

Conclusion: The lymphocyte subset repertoires in the DM patients were biased, and were associated with the presence of ILD and disease activity of DM.

Acknowledgements

We thank Tsubasa Okano and Motoi Yamashita for technical assistance and Shinichi Uchida for encouragement.

This study was supported by grants from the Ministry of Education, Culture, Sports, Science, and Technology of Japan and the Ministry of Health, Labour, and Welfare of Japan.

Disclosure statement

HK has received consulting fees, speaking fees, honoraria, and/or research grants from Astellas, Chugai, Japan Blood Products Organization, Mitsubishi-Tanabe, Pfizer, Ono, and Bristol-Myers. The other authors have no potential conflicts of interest to report.

Supporting Information

Additional Supporting Information may be found in the online version of this article.

Supplementary table S1. Comparison of lymphocyte subset proportions according to the presence of ILD.

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