期刊
NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY
卷 45, 期 5, 页码 495-512出版社
WILEY
DOI: 10.1111/nan.12528
关键词
anti-SRP; dermatomyositis; immune mediated necrotizing myopathy; JDM score tool; polymyositis; principal component analysis
资金
- Wellcome Trust UK [085860]
- Action Medical Research UK [SP4252]
- Myositis Support Group UK
- Arthritis Research UK [14518, 20164]
- Great Ormond Street Children's Charity [V1268]
- National Institute for Health Research (NIHR) Translational Research Collaboration (TRC) Rare Diseases
- NIHR Biomedical Research Centre at Great Ormond Street Hospital for Children NHS Foundation Trust
- GOS Institute of Child Health University College London (UCL)
- Great Ormond Street Children's Charity
- NIHR Biomedical Research Centre at Great Ormond Street Hospital
- Henry Smith Charity
- NIHR's Rare Diseases Translational Research Collaboration
Aim Juvenile idiopathic inflammatory myopathies have been recently reclassified into clinico-serological subgroups. Myopathological correlates of the subgroups are incompletely understood. Methods We studied muscle biopsies from 101 children with clinically and serologically defined juvenile idiopathic inflammatory myopathies from the UK JDM Cohort and Biomarker Study by applying the international JDM score tool, myopathological review and C5b-9 complement analysis. Results Autoantibody data were available for 90/101 cases with 18/90 cases positive for anti-TIF1 gamma, 15/90 anti-NXP2, 11/90 anti-MDA5, 5/90 anti-Mi2 and 6/90 anti-PmScl. JDM biopsy severity scores were consistently low in the anti-MDA5 group, high in the anti-Mi2 group, and widely distributed in the other groups. Biopsies were classified histologically as perifascicular atrophy (22/101), macrophage-rich necrosis (6/101), scattered necrosis (2/101), clustered necrosis (2/101), inflammatory fibre invasion (2/101), chronic myopathic change (1/101), diffuse endomysial macrophage infiltrates (40/101) and minimal change (24/101). MDA5 cases segregated with the minimal change group and showed no capillary C5b-9-deposition. The Mi2 group displayed high severity scores and a tendency towards sarcolemmal complement deposition. NXP2 and TIF1 gamma groups showed a variety of pathologies with a high proportion of diffuse endomysial macrophage infiltrates and a high proportion of capillary C5b-9 deposition. Conclusion We have shown that juvenile idiopathic inflammatory myopathies have a spectrum of histopathological phenotypes and show distinct complement attack complex deposition patterns. Both correlate in some cases with the serological subtypes. Most cases do not show typical histological features associated with dermatomyositis (e.g. perifascicular atrophy). In contrast, more than half show relatively mild histopathological changes.
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