Article ; Online: Myxovirus resistance protein A (MxA) expression in myositides: Sarcoplasmic expression is common in both dermatomyositis and lupus myositis.
2024 Volume 69, Issue 5, Page(s) 548–555
Abstract: Introduction/aims: Myxovirus resistance protein A (MxA) is a type I interferon (IFN1) pathway activation marker and MxA sarcoplasmic expression is currently recognized as a highly specific marker for dermatomyositis (DM). However, we have frequently ... ...
Abstract | Introduction/aims: Myxovirus resistance protein A (MxA) is a type I interferon (IFN1) pathway activation marker and MxA sarcoplasmic expression is currently recognized as a highly specific marker for dermatomyositis (DM). However, we have frequently observed endothelial tubuloreticular inclusions (TRI), another surrogate IFN1 activation marker, in a variety of overlap myositides. The aim of this study was to examine MxA expression in those myositides. Methods: We retrospectively performed MxA immunostaining on a wide range of myositides. Results: MxA sarcoplasmic expression was present in DM (94.4%, 17/18), active lupus myositis (LM, 80%,16/20), inactive LM (36%, 4/11), antisynthetase syndrome (ASyS, 20%, 2/10), systemic sclerosis (13%, 2/15), Sjogren's syndrome (7.7%, 1/13), and human immunodeficiency virus (HIV) myositis (5.6%, 1/18) and was absent in immune-mediated necrotizing myopathy (IMNM, 0/16) and hydroxychloroquine myopathy (0/5). The sensitivity and specificity of MxA sarcoplasmic expression for LM and DM combined compared with all other myositides were 84.6% (95% CI: 69.5-94.1) and 92.1 (95% CI: 83.6-97.0), respectively, and superior to TRIs. MxA capillary expression was nonspecific. Histologically, 35% of LM cases demonstrated a unique panfascicular necrotizing myopathy pattern. The remainder of the LM cases had significant morphological overlap with DM/ASyS (20%), IMNM (20%), or polymyositis (15%). Discussion: MxA sarcoplasmic expression is highly prevalent in LM and DM and is a useful marker in differentiating DM and LM from other myositides. LM can manifest in various pathology patterns that need to be differentiated from DM, IMNM, ASyS, and polymyositis. |
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MeSH term(s) | Humans ; Biomarkers ; Dermatomyositis/pathology ; Muscular Diseases ; Myositis/pathology ; Orthomyxoviridae ; Polymyositis/pathology ; Retrospective Studies |
Chemical Substances | Biomarkers |
Language | English |
Publishing date | 2024-02-19 |
Publishing country | United States |
Document type | Journal Article |
ZDB-ID | 438353-9 |
ISSN | 1097-4598 ; 0148-639X |
ISSN (online) | 1097-4598 |
ISSN | 0148-639X |
DOI | 10.1002/mus.28066 |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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