Perimysial microarteriopathy in dermatomyositis with anti‐nuclear matrix protein‐2 antibodies

• DOI: 10.1111/ene.14097
• Publication Date: 2019-10-01T06:05:13Z
• Authors: Y. Liu; Y. Zheng; Q. Gang; Z. Xie; Y. Jin; X. Zhang; X. Deng; H. Hao; F. Gao; Z. Zhang; H. Xiong; W. Zhang; Z. Wang; Y. Yuan
• Keywords: Sarcoplasm; Desmin

Background and purpose Dermatomyositis (DM) with anti‐nuclear matrix protein‐2 (NXP‐2) antibodies usually shows multifocal ischaemic lesions in muscle. Here, we aimed to investigate the microarteriopathy underlying muscle ischaemia anti‐NXP‐2‐positive DM. Methods A total of 16 patients diagnosed DM were investigated by biopsy. 13 other myositis‐specific 11 normal controls included for comparison. Immunofluorescence assays performed localize endothelial cells, smooth cells pericytes, determine myofibers microvessels vascular growth factor myxovirus resistance protein (MxA). Electron microscopy was carried out assess ultrastructure alterations. Results Subcutaneous edema, severe weakness dysphagia together elevated creatine kinase, D‐dimer triglyceride levels, decreased albumin levels found Muscle regional perifascicular atrophy, microinfarcts focal punched‐out vacuoles. The density arterioles higher ( P ＜ 0.05). Perimysial thickened wall, thrombosis lipid accumulation wall diseased perimysial arterioles. frequency < Sarcoplasmic MxA expression observed lesions. present pericytes. confirmed damaged capillaries tubuloreticular structures. Conclusions Our research suggested that most commonly involved DM, which led ischaemia.