NDLI: Muscle Biopsy Findings in Combination With Myositis-Specific Autoantibodies Aid Prediction of Outcomes in Juvenile Dermatomyositis.

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Deakin, Claire T. Yasin, Shireena A. Simou, Stefania Arnold, Katie A. Tansley, Sarah L. Betteridge, Zoe E. McHugh, Neil J. Varsani, Hemlata Holton, Janice L. Jacques, Thomas S. Pilkington, Clarissa A. Nistala, Kiran Wedderburn, Lucy R.
Description	Country affiliation: United kingdom Author Affiliation: Deakin CT ( University College London, London, UK.); Yasin SA ( University College London, London, UK.); Simou S ( University College London, London, UK.); Arnold KA ( University College London, London, UK.); Tansley SL ( University of Bath, Bath, UK.); Betteridge ZE ( University of Bath, Bath, UK.); McHugh NJ ( University of Bath, Bath, UK.); Varsani H ( University College London, London, UK.); Holton JL ( University College London, London, UK.); Jacques TS ( University College London, London, UK.); Pilkington CA ( Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.); Nistala K ( Great Ormond Street Hospital for Children NHS Foundation Trust and University College London, London, UK.); Wedderburn LR ( University College London, Great Ormond Street Hospital for Children NHS Foundation Trust, and University College London Hospitals, London, UK. l.wedderburn@ucl.ac.uk.)
Abstract	OBJECTIVE: Juvenile dermatomyositis (DM) is a rare and severe autoimmune condition characterized by rash and proximal muscle weakness. While some patients respond to standard treatment, others do not. This study was carried out to investigate whether histopathologic findings and myositis-specific autoantibodies (MSAs) have prognostic significance in juvenile DM. METHODS: Muscle biopsy samples (n = 101) from patients in the UK Juvenile Dermatomyositis Cohort and Biomarker Study were stained, analyzed, and scored for severity of histopathologic features. In addition, autoantibodies were measured in the serum or plasma of patients (n = 90) and longitudinal clinical data were collected (median duration of follow-up 4.9 years). Long-term treatment status (on or off medication over time) was modeled using generalized estimating equations. RESULTS: Muscle biopsy scores differed according to MSA subgroup. When the effects of MSA subgroup were accounted for, increased severity of muscle histopathologic features was predictive of an increased risk of remaining on treatment over time: for the global pathology score (histopathologist's visual analog scale [hVAS] score), 1.48-fold higher odds (95% confidence interval [95% CI] 1.12-1.96; P = 0.0058), and for the total biopsy score (determined with the standardized score tool), 1.10-fold higher odds (95% CI 1.01-1.21; P = 0.038). A protective effect was identified in patients with anti-Mi-2 autoantibodies, in whom the odds of remaining on treatment were 7.06-fold lower (95% CI 1.41-35.36; P = 0.018) despite muscle biopsy scores indicating more severe disease. In patients with anti-nuclear matrix protein 2 autoantibodies, anti-transcription intermediary factor 1Î³ autoantibodies, or no detectable autoantibody, increased histopathologic severity alone, without adjustment for the effect of MSA subtype, was predictive of the risk of remaining on treatment: for the hVAS global pathology score, 1.61-fold higher odds (95% CI 1.16-2.22; P = 0.004), and for the total biopsy score, 1.13-fold higher odds (95% CI 1.03-1.24; P = 0.013). CONCLUSION: Histopathologic severity, in combination with MSA subtype, is predictive of the risk of remaining on treatment in patients with juvenile DM and may be useful for discussing probable treatment length with parents and patients. Understanding these associations may identify patients at greater risk of severe disease.
File Format	HTM / HTML
ISSN	23265191
Issue Number	11
Journal	Arthritis & Rheumatology
Volume Number	68
e-ISSN	23265205
Language	English
Publisher	Wiley
Publisher Date	2016-11-01
Publisher Place	United States
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	Discipline Rheumatology
Content Type	Text
Resource Type	Article
Subject	Immunology and Allergy Immunology Rheumatology

Sl.	Authority	Responsibilities	Communication Details
1	Ministry of Education (GoI), Department of Higher Education	Sanctioning Authority	https://www.education.gov.in/ict-initiatives
2	Indian Institute of Technology Kharagpur	Host Institute of the Project: The host institute of the project is responsible for providing infrastructure support and hosting the project	https://www.iitkgp.ac.in
3	National Digital Library of India Office, Indian Institute of Technology Kharagpur	The administrative and infrastructural headquarters of the project	Dr. B. Sutradhar bsutra@ndl.gov.in
4	Project PI / Joint PI	Principal Investigator and Joint Principal Investigators of the project	Dr. B. Sutradhar bsutra@ndl.gov.in Prof. Saswat Chakrabarti will be added soon
5	Website/Portal (Helpdesk)	Queries regarding NDLI and its services	support@ndl.gov.in
6	Contents and Copyright Issues	Queries related to content curation and copyright issues	content@ndl.gov.in
7	National Digital Library of India Club (NDLI Club)	Queries related to NDLI Club formation, support, user awareness program, seminar/symposium, collaboration, social media, promotion, and outreach	clubsupport@ndl.gov.in
8	Digital Preservation Centre (DPC)	Assistance with digitizing and archiving copyright-free printed books	dpc@ndl.gov.in
9	IDR Setup or Support	Queries related to establishment and support of Institutional Digital Repository (IDR) and IDR workshops	idr@ndl.gov.in