Milman N, Hoffmann A L, Byg K E
Department of Pulmonary Medicine, Naestved Hospital, Denmark.
Acta Paediatr. 1998 Aug;87(8):871-8. doi: 10.1080/080352598750013662.
This paper reviews current knowledge of childhood sarcoidosis with regard to the epidemiology in Danes, clinical presentation, diagnostic procedures, treatment and prognosis. Sarcoidosis is a granulomatous disease of unknown aetiology, with multiorgan involvement. The diagnosis is confirmed by the demonstration of epitheloid cell granulomas in tissue biopsy specimens. During the period 1980-92, three cases of childhood sarcoidosis were recorded in Copenhagen County, which has a total population of 610,000. The approximate incidence of clinically recognized sarcoidosis in Danish children younger than 15 y of age was 0.22-0.27/100,000 children per year, corresponding to approximately three new cases in Denmark each year. The true incidence is unknown, since the disease is often asymptomatic and resolves without a clinical diagnosis being made. In children younger than 5 y of age, the disease is characterized by involvement of skin, eyes and joints, whereas in older children involvement of lungs, lymph nodes and eyes predominate. The mainstay of treatment consists of oral corticosteroids. The risk/benefit ratio of using long-term corticosteroids needs to be evaluated in each individual patient. Some patients may benefit from additional therapy with methotrexate. The long-term prognosis is not well established, but it seems to be poorer in children younger than 5 y. Older children appear to have as favourable a prognosis as young adults.
本文综述了丹麦儿童结节病在流行病学、临床表现、诊断方法、治疗及预后方面的现有知识。结节病是一种病因不明的肉芽肿性疾病，可累及多个器官。通过组织活检标本中出现上皮样细胞肉芽肿来确诊。1980年至1992年期间，哥本哈根郡共记录了3例儿童结节病病例，该郡总人口为61万。丹麦15岁以下儿童临床确诊结节病的年发病率约为0.22 - 0.27/10万儿童，即丹麦每年约有3例新发病例。由于该病常无症状且未经临床诊断即可自行缓解，故实际发病率尚不清楚。5岁以下儿童的疾病特征为皮肤、眼睛和关节受累，而大龄儿童则以肺部、淋巴结和眼睛受累为主。治疗的主要方法是口服糖皮质激素。每位患者都需要评估长期使用糖皮质激素的风险/获益比。一些患者可能从甲氨蝶呤的额外治疗中获益。长期预后尚不明确，但5岁以下儿童的预后似乎较差。大龄儿童的预后似乎与年轻成年人一样良好。
