PD1 and PDL2 axis confers T cell exhaustion in anti-SRP+ and anti-HMGCR+ myopathies

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Exposure to persistent antigen as in Immune-mediated necrotizing myopathies (IMNM) may lead to defective T cell function called ‘exhaustion’, which is crucially controlled by programmed cell death protein 1 (PD1) and its cognate ligands PD1L/PD2L. These molecules have recently been successfully used as immune checkpoint blockers in certain cancers. However, therapy with these may lead to increased autoimmune responses with development of e.g. myocarditis or myositis, pointing to relevance of this pathway in muscle inflammation.

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