de Mâcedo Patrícia Andrade, Shinjo Samuel Katsuyuki, Goldenstein-Schainberg Cláudia
Serviço de Reumatologia, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brasil.
Acta Reumatol Port. 2008 Jul-Sep;33(3):289-97.
Juvenile scleroderma is a rare childhood condition characterized by fibrosis of the skin and internal organs. Clinical manifestations of childhood scleroderma are different from adult disease and early recognition, correct classification and treatment can improve long-term outcome. This review explores the most recent actualizations on clinical manifestations, classification criteria, treatment options and prognosis of juvenile scleroderma. There are two main forms of the disease: localized scleroderma and systemic sclerosis. Localized scleroderma is the most common form in children and mostly restricted to the skin. Juvenile diffuse systemic sclerosis is related to visceral involvement and cardiac disease which is the main cause of death in these patients. The outcome of juvenile systemic sclerosis is better compared with the adult form. Treatment remains a medical challenge and the EULAR task force proposed an approach to juvenile scleroderma treatment based on expert's opinion and guidelines used for the treatment of adults. Larger studies on childhood scleroderma are warranted.
青少年硬皮病是一种罕见的儿童疾病，其特征为皮肤和内脏器官纤维化。儿童硬皮病的临床表现与成人疾病不同，早期识别、正确分类和治疗可改善长期预后。本综述探讨了青少年硬皮病在临床表现、分类标准、治疗选择和预后方面的最新进展。该疾病主要有两种形式：局限性硬皮病和系统性硬化症。局限性硬皮病是儿童中最常见的形式，主要局限于皮肤。青少年弥漫性系统性硬化症与内脏受累及心脏疾病有关，而心脏疾病是这些患者的主要死因。与成人形式相比，青少年系统性硬化症的预后较好。治疗仍然是一项医学挑战，欧洲抗风湿病联盟（EULAR）特别工作组基于专家意见和成人治疗指南提出了一种青少年硬皮病的治疗方法。有必要开展关于儿童硬皮病的更大规模研究。
