Juvenile dermatomyositis: new insights and new treatment strategies

Abstract

Juvenile dermatomyositis (JDM) is a rare but complex and potentially lifethreatening autoimmune disease of childhood, primarily affecting proximal muscles and skin. Although the cause of JDM remains unknown it is clear that genetic and environmental influences play a role in the aetiology. In contrast to adults with dermatomyositis, children with JDM are more likely to have complications that are thought to indicate a vasculopathic process, such as severe skin disease, with ulceration or calcinosis, gut vasculopathy or central nervous system disease. New treatments are much needed and are becoming available and being tested through international multicentre trials. This review will focus on recent insights into pathogenesis, the assessment of the disease in children and the modern approach to its treatment.

Association of circulating follicular helper T cells with disease course of NMO spectrum disorders

“…It was also reported that IL-21 was increased in PBMCs from MG patients . Martin et al (2012) reported that the levels of circulating Tfh cells were increased in autoimmune thyroid diseases and CD4 + T cells from patients with Graves' disease or Hashimoto's thyroiditis displayed increased IL-21 secretion and expression following in vitro culture. In juvenile dermatomyositis, an increase of CXCR5 + CCR6 + Th17-like and CXCR5 + CXCR3 − CCR6 − Th2-like Tfh cells was detected, and the disease score (Luo et al, 2013;Zhu et al, 2012) and the plasmablast numbers correlated positively with these Tfh cells (Wong et al, 2010).…”

Treatment of Juvenile Dermatomyositis: An Update

“…With the advantage of recent therapeutic strategies and the development of new treatments, survival and outcomes have improved significantly with a reported mortality of <2% [8,9]. As JDM is rare, much of our knowledge on treatment is based on anecdotal experience or small case series.…”

Dermatomyositis/polymyositis and anti-TNF-α therapies: a literature review

“…The definitive diagnosis of PM requires the exclusion of DM and other inflammatory myopathies [1,2]. As patients affected by DM, PM patients have progressive proximal muscle weakness, which typically develops over weeks or months [1,2].…”

“…The cutaneous histological examination may be in many cases unspecific, because it may appear very similar to cutaneous lupus erythematosus [1][2][3].…”

“…As patients affected by DM, PM patients have progressive proximal muscle weakness, which typically develops over weeks or months [1,2].…”