Irish journal of medical science · 2008
Juvenile localised scleroderma: a retrospective review of response to systemic treatment.
СтатьяНазвание и аннотация: Перевод в очереди
Обработка статьи · этапы, ошибки и управление
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Cox D, O' Regan G, Collins S, Byrne A, Irvine A, Watson R
Аннотация
BACKGROUND: Juvenile localised scleroderma (JLS) is a rare connective tissue disorder in childhood. Most lesions are benign and self-limiting, but some progress to cause functional disabilities and cosmetic disfigurement. These lesions require systemic treatment, the mainstay of which is corticosteroids and methotrexate.
AIM: To report the experience of the use of systemic treatment in children with JLS in our department.
METHODS: We performed a retrospective chart review looking at the patients who received systemic treatment over 14 years and recorded their outcomes.
RESULTS: Ten children with aggressive JLS were treated with systemic immunosuppressive therapy. There was an 80% response to treatment. Three of the responders relapsed off treatment, but responded to a further course of therapy. Mean disease duration at diagnosis was 8 months. Treatment was generally well tolerated with few side effects.
CONCLUSION: Early recognition of aggressive localised scleroderma and appropriate referral is imperative for a good outcome.
AIM: To report the experience of the use of systemic treatment in children with JLS in our department.
METHODS: We performed a retrospective chart review looking at the patients who received systemic treatment over 14 years and recorded their outcomes.
RESULTS: Ten children with aggressive JLS were treated with systemic immunosuppressive therapy. There was an 80% response to treatment. Three of the responders relapsed off treatment, but responded to a further course of therapy. Mean disease duration at diagnosis was 8 months. Treatment was generally well tolerated with few side effects.
CONCLUSION: Early recognition of aggressive localised scleroderma and appropriate referral is imperative for a good outcome.