The composition of cellular infiltrates in anti-HMG-CoA reductase-associated myopathy

Muscle & Nerve
Tae ChungAndrew L Mammen

Abstract

To characterize cellular infiltrates in muscle biopsies from patients with anti-3-hydroxy-3-methyl-gulatryl-CoA reductase (HMGCR)-associated myopathy. Biopsies from 18 anti-HMGCR myopathy and 7 control dermatomyositis patients were analyzed. CD4+ and CD8+ T-cells were scattered within the endomysium in 50% of anti-HMGCR biopsies. All anti-HMGCR biopsies included increased endomysial and/or perivascular CD163+ M2 macrophages; CD11c+ M1 macrophages were present in 18.8%. CD123+ plasmacytoid dendritic (PD) cells were observed within the endomysium and perivascular spaces in 62.5% of anti-HMGCR biopsies. Membrane attack complex was deposited on endothelial cells in 50% and on the sarcolemma of nonnecrotic muscle fibers in 85.7% of anti-HMGCR cases. Major histocompatibility complex class I antigen was up-regulated in 87.5% of the anti-HMGCR cases. In addition to necrosis, scattered CD4+, CD8+, and PD cells are characteristic of anti-HMGCR myopathy. Predominant M2 polarization suggests infiltrating macrophages are more likely to be involved with tissue repair than destruction.

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Citations

Feb 18, 2016·The New England Journal of Medicine·Andrew L Mammen
Aug 14, 2015·International Journal of Molecular Sciences·Boel De Paepe, Jana Zschüntzsch
Oct 23, 2015·Expert Review of Clinical Immunology·Albert Selva-O'CallaghanIago Pinal-Fernandez
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