Abstract
The International Juvenile Dermatomyositis (JDM) Biopsy Consensus Group has previously published and validated a scoring tool for assessment of the severity of pathological change in biopsy specimens from patients with suspected or proven JDM. This tool assesses histological features characteristic of JDM, organised into four domains (inflammatory, vascular, muscle fibre and connective tissue). The score tool includes the assessment of CD3+ and CD68+ inflammatory cells. Given that JDM is a disease characterised by production of autoantibodies and some patients respond to anti-B cell therapy we hypothesise that B cells play an important role in the pathogenesis of the disease.
Highlights
The International Juvenile Dermatomyositis (JDM) Biopsy Consensus Group has previously published and validated a scoring tool for assessment of the severity of pathological change in biopsy specimens from patients with suspected or proven JDM
Given that JDM is a disease characterised by production of autoantibodies and some patients respond to anti-biopsy was stained for cells expressing CD20 (B cells) therapy we hypothesise that B cells play an important role in the pathogenesis of the disease
Among those biopsies that contained CD138+ plasma cells, the CD138+ score was correlated with the score for CD20+ cells (r=0.89; p=0.026), the score for CD3+ infiltrating cells (r=1.0; p
Summary
The International Juvenile Dermatomyositis (JDM) Biopsy Consensus Group has previously published and validated a scoring tool for assessment of the severity of pathological change in biopsy specimens from patients with suspected or proven JDM. This tool assesses histological features characteristic of JDM, organised into four domains (inflammatory, vascular, muscle fibre and connective tissue). The score tool includes the assessment of CD3+ and CD68+ inflammatory cells. Given that JDM is a disease characterised by production of autoantibodies and some patients respond to anti-B cell therapy we hypothesise that B cells play an important role in the pathogenesis of the disease
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