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Biopsy pathology in a large cohort of juvenile dermatomyositis is heterogeneous and, for the most part, independent of autoantibody phenotype

Published online by Cambridge University Press:  30 January 2017

Shireena A Yasin ,
Peter W Schutz ,
Claire T Deakin ,
Erdal Sag ,
Hemlata Varsani ,
Stephanie Simou ,
Sarah Tansley ,
Neil McHugh ,
Janice L Holton  and
Lucy R Wedderburn
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Shireena A Yasin
Affiliation:
Infection, Immunity, Inflammation Programme, UCL GOS Institute of Child Health, London, UK
Peter W Schutz
Affiliation:
Infection, Immunity, Inflammation Programme, UCL GOS Institute of Child Health, London, UK Division of Neuropathology, UCL Institute of Neurology, London, UK
Claire T Deakin
Affiliation:
Infection, Immunity, Inflammation Programme, UCL GOS Institute of Child Health, London, UK
Erdal Sag
Affiliation:
Infection, Immunity, Inflammation Programme, UCL GOS Institute of Child Health, London, UK
Hemlata Varsani
Affiliation:
Infection, Immunity, Inflammation Programme, UCL GOS Institute of Child Health, London, UK
Stephanie Simou
Affiliation:
Infection, Immunity, Inflammation Programme, UCL GOS Institute of Child Health, London, UK
Sarah Tansley
Affiliation:
Royal National Hospital for Rheumatic Diseases, Royal United Hospitals Foundation Trust, Bath; and University of Bath, UK
Neil McHugh
Affiliation:
Royal National Hospital for Rheumatic Diseases, Royal United Hospitals Foundation Trust, Bath; and University of Bath, UK
Janice L Holton
Affiliation:
Division of Neuropathology, UCL Institute of Neurology, London, UK MRC Centre for Neuromuscular Diseases, UCL Institute of Neurology, London, UK
Lucy R Wedderburn
Affiliation:
Infection, Immunity, Inflammation Programme, UCL GOS Institute of Child Health, London, UK Rheumatology Unit, Great Ormond Street Hospital for Children, London, UK
Thomas S Jacques
Affiliation:
Developmental Biology and Cancer Programme, UCL Institute of Child Health and Department of Histopathology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK
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Abstract

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Type
Abstracts
Information
Canadian Journal of Neurological Sciences , Volume 44 , Supplement S1: ABSTRACTS: 56th Annual Canadian Association of Neuropathologists Meeting , February 2017 , pp. S6
Copyright
Copyright © The Canadian Journal of Neurological Sciences Inc. 2017 

BACKGROUND

Juvenile dermatomyositis has come to encompass several subtypes based on an emerging correlation between autoantibodies and clinical presentation. We hypothesised that myopathological findings may align with clinico-serological subtypes, potentially indicating differences in pathogenesis.

METHODS

We studied a large cohort of 101 muscle biopsies from JDM patients in the UK JDM Cohort and Biomarker Study using the international JDM score tool and performing histological analysis of dominant fiber pathology. Autoantibody data were available for the majority of patients and were correlated with histological findings.

RESULTS

Major autoantibody groups in our cohort were anti-TIF1γ (18/101), -NXP2 (15/101), -MDA5 (11/101), -Mi2 (5/101), and -PmScl (6/101). JDM biopsy severity scores varied within antibody groups except for MDA5 with consistently low, and Mi2 with consistently high scores. Dominant fiber pathology was grouped under 8 descriptive labels (minimal change (24/101), diffuse endomysial macrophage infiltrates (40/101), perifascicular atrophy (22/101), macrophage rich necrosis (6/101), scattered necrosis (2/101), clustered necrosis (2/101), inflammatory fiber invasion (2/101), chronic myopathic change (1/101)). All major autoantibody groups showed a mix of fiber pathologies with the exception of MDA5, which consisted predominantly of minimal change biopsies.

CONCLUSION

JDM patients demonstrate a range of muscle biopsy findings in our cohort with perfascicular atrophy represented in only about one third of biopsies. Dominant fiber pathology or severity scores do not clearly predict autoantibody groups. Heterogeneity of muscle histology in JDM is not fully understood but may indicate differences in activation of inflammatory signaling pathways in muscle between patients.