Expression of myxovirus-resistance protein A: a possible marker of muscle disease activity and autoantibody specificities in juvenile dermatomyositis

Neuropathology and Applied Neurobiology
S SoponkanapornThomas S Jacques

Abstract

To evaluate the relationship between expression of myxovirus-resistance protein A (MxA) protein on muscle biopsies by immunohistochemistry and disease activity in juvenile dermatomyositis (JDM) patients. Also, another aim was to investigate whether the expression of MxA is related with myositis-specific autoantibodies (MSA) status in JDM patients. 103 patients (median aged 6.3, interquartile range 0.5-15.9) enrolled in the Juvenile Dermatomyositis Cohort and Biomarker Study (JDCBS). Muscle biopsies were stained with MxA and scored. Clinical data at initial presentation were collected and autoantibodies were analysed. Multiple linear regression analysis was performed to estimate the association between MxA expression on muscle fibres and muscle disease activity, and MSA status. Expression of MxA protein on JDM samples was identified in 61.2%. There was a significant association between MxA scores and Childhood Myositis Assessment Scale (CMAS) (P = 0.002), and Manual Muscle Testing of Eight Muscles (MMT8) (P = 0.026). CMAS and MMT8 scores were significantly lower in the group of patients with strong MxA expression. MxA scores differed according to MSA subgroups (P = 0.002). Patients with positive nuclear matrix protein 2 autoanti...Continue Reading

References

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Citations

Aug 28, 2020·Current Opinion in Neurology·Jantima TanboonIchizo Nishino
Oct 28, 2020·Rheumatology International·Erdal SagBeril Talim
Dec 6, 2020·Neurology·Jantima TanboonIchizo Nishino
Jul 3, 2021·Current Rheumatology Reports·Akinori UruhaWerner Stenzel
Jul 8, 2021·Current Opinion in Rheumatology·Hanna Kim

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Methods Mentioned

BETA
biopsies
biopsy
light microscopy

Software Mentioned

Fiji
SPSS Statistics

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