Journal: Acta Neuropathologica
Article Title: Brachio-cervical inflammatory myopathy: multilevel clinical, histopathological and multi-omic analyses of a syndrome variably associated with systemic sclerosis
doi: 10.1007/s00401-026-03006-5
Figure Lengend Snippet: Immune pathology, B-cell characterization, and tertiary lymphoid organ-related features. a Diffuse relatively mild sarcolemmal MHC cl. 1 positivity and presence of many MHC cl. 1 positive lymphomonocytic cells in the endomysium, and in a B-cell cluster (arrow). MHC cl. 1 immunohistochemistry; original magnification ×200. b Mild and infrequent sarcolemmal MHC cl. 2 positivity and presence of many MHC cl. 2 positive lymphomonocytic cells in the endomysium, and in a B-cell cluster (arrow). MHC cl. 2 immunohistochemistry; original magnification ×200. c Sarcolemmal complement deposits (arrow) and presence of single necrotic myofibers (arrowhead) that show sarcoplasmic complement deposits. C5b-9 (MAC) immunohistochemistry; original magnification ×200. d Presence of numerous endomysial macrophages and occasional myophagocytoses. CD68 immunohistochemistry; original magnification ×200. e Scattered, individual endomysial T cells. CD8 immunohistochemistry; original magnification ×200. f Focally clustering B cells with a roundish appearance reminiscent of so-called tertiary lymphoid organs (TLOs). CD20 immunohistochemistry; original magnification ×200. g , h TLO-like immune cell clusters show compartmentalization with bcl2-positive B-cell nuclei at the margins and bcl6-positive nuclei in their (germinal) center. Bcl2 (G) and bcl6 (H) immunohistochemistry; original magnification ×600. i Numerous Ki67 + proliferating cells are focally clustering predominantly in germinal centers of TLOs. Ki67 immunohistochemistry; original magnification ×600. j A B-cell cluster contains some CD138-positive plasma cells. CD138 immunohistochemistry; original magnification ×200. k Similarly, those plasma cells and a few B cells are identified by nuclear MUM1 positivity in the light zone of a germinal center. MUM1 immunohistochemistry; original magnification ×200. l CD38-positive plasmablasts are partly proliferating and show co-immunoreactivity with Ki67. CD38 AF488 (green) and Ki67 Cy3 (red) immunofluorescence; original magnification ×200. a – l Scale bar represents 100 µm
Article Snippet: The antibodies used for immunohistochemistry included C5b-9 (Dako, aE11, 1:200), CD8 (Dako, C8/144B, 1:100), CD20 (Dako, Ks 20.8 1:200), Ki-67 (Dako, Mib-1, 1:100), MxA (Millipore, MABF938, 1:100), CD38 (Novocastra SPC32, 1:100), MUM1 (Dako, MUM1P, 1:50), CD45 (Dako, 2B11, 1:400), CD56 (Serotec/MCA591 ERIC-1, 1:400), CD68 (Dako, EBM11, 1:100), MHC class I (Dako, w6/32, 1:1000), MHC class II (Dako, CR3/43, 1:100), and SQSTM1/p62 (Abcam, polyclonal, 1:100).
Techniques: Cell Characterization, Immunohistochemistry, Clinical Proteomics, Immunofluorescence