The Vasculopathy of Juvenile Dermatomyositis
Juvenile dermatomyositis (JDM) is a rare autoimmune disease mainly characterized by muscle and skin involvement. Vasculopathy is considered central to the pathogenesis of the disease. The exact nature of vasculopathy is not yet understood but it is a complex process with both an inflammatory and a n...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2018-10-01
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| Series: | Frontiers in Pediatrics |
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| Online Access: | https://www.frontiersin.org/article/10.3389/fped.2018.00284/full |
| _version_ | 1818475656369930240 |
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| author | Charalampia Papadopoulou Charalampia Papadopoulou Liza J. McCann |
| author_facet | Charalampia Papadopoulou Charalampia Papadopoulou Liza J. McCann |
| author_sort | Charalampia Papadopoulou |
| collection | DOAJ |
| description | Juvenile dermatomyositis (JDM) is a rare autoimmune disease mainly characterized by muscle and skin involvement. Vasculopathy is considered central to the pathogenesis of the disease. The exact nature of vasculopathy is not yet understood but it is a complex process with both an inflammatory and a non-inflammatory, occlusive component. Impaired function of JDM vasculature includes immune complex deposition, altered expression of cell adhesion molecules predominantly inducing Th17 cell infiltration, and endothelial cell dysfunction. Development of vasculopathy is associated with the severe extra-muscular manifestations of JDM, such as gastrointestinal and cardiac manifestations, interstitial lung disease, ulcerative skin disease or development of calcinosis, and portends a poor prognosis. Correlation of histopathological findings, autoantibodies, and extensive diagnostic workup represent key elements to the early detection of vasculopathic features and early aggressive treatment. Monitoring of vasculopathy remains challenging due to the lack of non-invasive biomarkers. Current treatment approaches provide variable benefit, but better understanding of the essential pathogenic mechanisms should help lead to improved outcomes. Whilst acknowledging that evidence is limited, this review aims to describe the vasculopathy of JDM in the context of pathophysiology, clinical features, and treatment of disease. |
| first_indexed | 2024-12-10T09:15:32Z |
| format | Article |
| id | doaj.art-777446a49cf6468e8307807ad51b59d8 |
| institution | Directory Open Access Journal |
| issn | 2296-2360 |
| language | English |
| last_indexed | 2024-12-10T09:15:32Z |
| publishDate | 2018-10-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Pediatrics |
| spelling | doaj.art-777446a49cf6468e8307807ad51b59d82022-12-22T01:54:53ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602018-10-01610.3389/fped.2018.00284411668The Vasculopathy of Juvenile DermatomyositisCharalampia Papadopoulou0Charalampia Papadopoulou1Liza J. McCann2Infection, Inflammation, and Rheumatology Section, UCL Great Ormond Street Institute of Child Health, London, United KingdomGreat Ormond Street Hospital NHS Foundation Trust, London, United KingdomDepartment of Pediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, United KingdomJuvenile dermatomyositis (JDM) is a rare autoimmune disease mainly characterized by muscle and skin involvement. Vasculopathy is considered central to the pathogenesis of the disease. The exact nature of vasculopathy is not yet understood but it is a complex process with both an inflammatory and a non-inflammatory, occlusive component. Impaired function of JDM vasculature includes immune complex deposition, altered expression of cell adhesion molecules predominantly inducing Th17 cell infiltration, and endothelial cell dysfunction. Development of vasculopathy is associated with the severe extra-muscular manifestations of JDM, such as gastrointestinal and cardiac manifestations, interstitial lung disease, ulcerative skin disease or development of calcinosis, and portends a poor prognosis. Correlation of histopathological findings, autoantibodies, and extensive diagnostic workup represent key elements to the early detection of vasculopathic features and early aggressive treatment. Monitoring of vasculopathy remains challenging due to the lack of non-invasive biomarkers. Current treatment approaches provide variable benefit, but better understanding of the essential pathogenic mechanisms should help lead to improved outcomes. Whilst acknowledging that evidence is limited, this review aims to describe the vasculopathy of JDM in the context of pathophysiology, clinical features, and treatment of disease.https://www.frontiersin.org/article/10.3389/fped.2018.00284/fullJuveniledermatomyositisvasculopathyantibodiespathophysiology |
| spellingShingle | Charalampia Papadopoulou Charalampia Papadopoulou Liza J. McCann The Vasculopathy of Juvenile Dermatomyositis Frontiers in Pediatrics Juvenile dermatomyositis vasculopathy antibodies pathophysiology |
| title | The Vasculopathy of Juvenile Dermatomyositis |
| title_full | The Vasculopathy of Juvenile Dermatomyositis |
| title_fullStr | The Vasculopathy of Juvenile Dermatomyositis |
| title_full_unstemmed | The Vasculopathy of Juvenile Dermatomyositis |
| title_short | The Vasculopathy of Juvenile Dermatomyositis |
| title_sort | vasculopathy of juvenile dermatomyositis |
| topic | Juvenile dermatomyositis vasculopathy antibodies pathophysiology |
| url | https://www.frontiersin.org/article/10.3389/fped.2018.00284/full |
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