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Muscle Biopsy Findings in Combination With Myositis-Specific Autoantibodies Aid Prediction of Outcomes in Juvenile Dermatomyositis.
- Source :
-
Arthritis & rheumatology (Hoboken, N.J.) [Arthritis Rheumatol] 2016 Nov; Vol. 68 (11), pp. 2806-2816. Date of Electronic Publication: 2016 Oct 09. - Publication Year :
- 2016
-
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.<br />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.<br />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 1γ 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).<br />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.<br /> (© 2016 The Authors. Arthritis & Rheumatology published by Wiley Periodicals, Inc. on behalf of the American College of Rheumatology.)
- Subjects :
- Adrenal Cortex Hormones therapeutic use
Child
Child, Preschool
Cohort Studies
DNA Topoisomerases immunology
DNA-Binding Proteins immunology
Dermatomyositis drug therapy
Dermatomyositis immunology
Female
Humans
Immunosuppressive Agents therapeutic use
Interferon-Induced Helicase, IFIH1 immunology
Longitudinal Studies
Male
Methotrexate therapeutic use
Odds Ratio
Prognosis
Protective Factors
Risk Factors
Severity of Illness Index
Signal Recognition Particle immunology
Threonine-tRNA Ligase immunology
Transcription Factors immunology
United Kingdom
Autoantibodies immunology
Dermatomyositis pathology
Quadriceps Muscle pathology
Subjects
Details
- Language :
- English
- ISSN :
- 2326-5205
- Volume :
- 68
- Issue :
- 11
- Database :
- MEDLINE
- Journal :
- Arthritis & rheumatology (Hoboken, N.J.)
- Publication Type :
- Academic Journal
- Accession number :
- 27214289
- Full Text :
- https://doi.org/10.1002/art.39753