Treatment of Juvenile Dermatomyositis: An Update

Papadopoulou, Charalampia; Wedderburn, Lucy R.

doi:10.1007/s40272-017-0240-6

Cited by 31 publications

(41 citation statements)

References 126 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Children are taught to apply high-factor sunscreen daily as ultraviolet light can trigger JDM flares (15). Long-term corticosteroids are associated with a number of complications, and children may be co-prescribed calcium and vitamin D to reduce risk of osteoporosis (32) and gastro-protective cover to reduce risk of gastrointestinal bleeding and perforation (8).…”

Section: [B] Adjunctive Treatmentsmentioning

confidence: 99%

“…Reduced physical activity can result in deconditioning, obesity, cardiovascular disease and metabolic syndrome (8). This is of particular concern in children taking long-term corticosteroids and in subsets of JDM with lipodystrophy (associated with insulin resistance, diabetes mellitus and hyperlipidaemia).…”

Section: [B] Physiotherapymentioning

confidence: 99%

“…Janus Kinase (JAK) acts downstream of IFN, and JAK inhibitors have been used to good effect in children with interferonopathies (some of these children bear clinical features similar to JDM)(8). Use of JAK inhibitors in JDM is being trialed in North America and Europe, and it is thought that children with evidence of chronic endothelial injury may be most suitable for this therapy.Cytotoxic T cell Lymphocyte Antigen-4 (CTLA-4), CD28 and CD86 are expressed on muscle cells or inflammatory cells within muscles in inflammatory myopathies(8). Abatacept is a CTLA-4 fusion protein that binds to CD80/CD86 present on antigen presenting cells, preventing binding of CD80/CD86 to CD28 on T cells, inhibiting T cell activation.…”

mentioning

confidence: 99%

“…Currently, a trial of abatacept in refractory moderately active JDM is in progress in the United States.Serum IL-6 has been correlated with disease activity in JDM(68). Case reports of adults with polymyositis and an adult with overlap syndrome identify improvement of skin and muscle disease with tocilizumab (IL-6 receptor antibody)(8). To date, experience of tocilizumab in JDM is very limited.…”

mentioning

confidence: 99%

“…Evidence for mechanisms contributing to juvenile dermatomyositis pathogenesis (3,4,8). [Footnote -Figure 1 ]: pDC: plasmacytoid dendritic cell; c': complement; Treg: regulatory T cell; IL: interleukin; MCP: monocyte chemoattractant protein; IFN: interferon; MHC: major histocompatibility complex; CXCL: Chemokine (C-X-C motif) ligand; MRP: myeloid-related protein; TLR: toll-like receptor; MAC: membrane attack complex; ICAM: intercellular adhesion molecule; VCAM: vascular cell adhesion molecule Flow chart adapted from Single Hub and Access point for Paediatric Rheumatology in Europe (SHARE) recommendations for treatment of juvenile dermatomyositis.…”

mentioning

confidence: 99%

See 4 more Smart Citations

Juvenile dermatomyositis: Latest advances

Wedderburn

McCann

2017

Best Practice & Research Clinical Rheumatology

Self Cite

View full text Add to dashboard Cite

Registries and biobanks for juvenile dermatomyositis (JDM) have generated statistical power to help understand pathogenesis and determine treatment and long-term outcomes in this rare and heterogeneous disease. Genotype, autoantibodies, muscle histology and early clinical features may predict prognosis and guide personalised treatment. While corticosteroids and disease-modifying anti-rheumatic drugs improve outcomes, there remain children who experience refractory disease. Ongoing research into the aberrant immune response and novel biological targets is necessary. Best practice guidelines promote prompt stepwise treatment, and there is growing appreciation of the role of exercise in improving prognosis. Validated tools standardise assessment of disease activity and damage in musculoskeletal, mucocutaneous, pulmonary, cardiac, gastrointestinal and endocrine systems. Recently, an internationally agreed dataset for JDM has been defined for clinical practice and incorporation into registries. In the future, with bigger datasets, statistical models may guide stratification for personalised medicine and discern the most relevant outcome markers for research.

show abstract

Section: [B] Adjunctive Treatmentsmentioning

confidence: 99%

Section: [B] Physiotherapymentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

See 3 more Smart Citations

Juvenile dermatomyositis: Latest advances

Wedderburn

McCann

2017

Best Practice & Research Clinical Rheumatology

Self Cite

View full text Add to dashboard Cite

show abstract

Growth and Puberty in Juvenile Dermatomyositis: A Longitudinal Cohort Study

Nordal

Pistorio

Rygg

et al. 2020

Arthritis Care & Research

View full text Add to dashboard Cite

Objective. To study growth and puberty in a multinational longitudinal prospective cohort of children with juvenile dermatomyositis (DM).Methods. Children from 31 countries who were ages <18 years and had juvenile DM in active phase were studied, and analyses of height, weight, and pubertal development were conducted in those who had follow-up visits during a 2-year period and for whom anthropometric data was available.Results. A total of 196 of 275 children (71%) were included. We found a significant reduction in parent-adjusted height Z score over time in female patients (P < 0.0001) and male patients (P = 0.001), but with catch-up growth at the final study visit. Median body mass index Z score peaked at 6 months (P < 0.0001) and was still significantly above baseline at the final study visit, which was at a median of 26 months after baseline (P = 0.007), with no difference between sexes. Female patients with a disease duration ≥12 months after onset had significantly lower parent-adjusted height Z score (P = 0.002) and no 2-year catch-up growth. At the final study visit, growth failure was seen in 20 of 97 female patients (21%) and in 11 of 73 male patients (15%). Height deflection (∆height Z score less than -0.25/year) was observed in 29 of 116 female patients (25%) and 25 of 80 male patients (31.3%). Delayed puberty was seen in 20 of 55 female patients (36.4%) and in 11 of 31 male patients (35.5%). Children in early pubertal stage at baseline had the highest risk of growth failure.Conclusion. Juvenile DM in the active phase and/or its treatment has a significant impact on growth and puberty in affected children. Children with recent onset of puberty or previous growth failure have the highest risk of delayed pubertal development and further growth retardation.

show abstract