Table 1.
IM subtype | Muscle biopsy | Cellular infiltrate | Associated autoantibodies | References |
---|---|---|---|---|
Dermatomyositis | Perimysial and perivascular inflammation; perivascular atrophy; MAC deposition; MHC-I and II expression | CD4+ T cells; macrophages; B cells; plasma cells; BDCA-2 + plasmacytoid dendritic cells; CD8+ CD28−/− and CD4+ CD28n−/− T cells; mast cells; γ/Δ T cells | Anti-Mi 2 | 3,10,11,21–26 |
Anti-TIF1y | ||||
Anti-MDA5/anti-CADM-140 | ||||
Anti-NXP-2 | ||||
Anti-SAE | ||||
Polymyositis | Endomysial inflammation; MHC-I and II expression | CD8+ T cells; macrophages; myeloid dendritic cells; CD68+ macrophages; BDCA-1 + myeloid dendritic cells; CD8+ CD28−/− and CD4+ CD28n−/− T cells; mast cells; γ/Δ T cells | Anti-tRNA synthetases (e.g. anti-Jo) | 3,10,11,21,25–27 |
Anti-SRP | ||||
Inclusion body myositis | Endomysial inflammation; rimmed vacuoles; protein aggregation, inclusions; MHC-I and II expression | CD8+ T cells; macrophages; myeloid dendritic cells; CD68+ macrophages; BDCA-1 + myeloid dendritic cells | Anti-cN1A | 3,21,25–27 |
Immune-mediated necrotizing myopathies | Endomysial and perimysial inflammation; MAC, MHC-I expression | CD68+ macrophages; few CD4+, CD8+ T cells, B cells | Anti-HMGCR | 25,28–31 |
Anti-SRP | ||||
Anti-tRNA synthetases |
Adapted and modified from Dimachkie [25]. MAC = membrane attack complex; MHC = major histocompatibility complex; BDCA = blood dendritic cell antigen; Tif = and transcriptional intermediary factor; MDA = melanoma differentiation-associated protein; NXP = nuclear matrix protein; CADM = clinically amyopathic dermatomyositis; SAE = small ubiquitin-like modifier activating enzyme; SRP = signal recognition particle; HMGCR = 3-hydroxymethyl glutaryl co-enzyme A reductase.