Shetty A K, Gedalia A
Department of Pediatrics, Louisiana State University Medical Center, New Orleans 70112, USA.
Clin Pediatr (Phila). 1998 Dec;37(12):707-17. doi: 10.1177/000992289803701201.
Childhood sarcoidosis is a rare multisystemic granulomatous disease of unknown etiology. The clinical presentation can vary greatly depending upon the organs involved. Two distinct forms of sarcoidosis exist in children. Older children usually present with a multisystem disease similar to the adult manifestation, with frequent hilar lymphadenopathy and pulmonary infiltration. Early-onset childhood sarcoidosis is a unique form of the disease characterized by the triad of rash, uveitis, and arthritis in patients presenting before age 4 years. The diagnosis of sarcoidosis is confirmed by demonstrating a typical noncaseating granuloma on a biopsy specimen. The current therapy of choice for childhood sarcoidosis with multisystem involvement is corticosteroids. Methotrexate given orally in low doses is effective and safe and has steroid-sparing properties.
儿童结节病是一种病因不明的罕见多系统肉芽肿性疾病。临床表现因受累器官不同而有很大差异。儿童结节病有两种不同形式。大龄儿童通常表现为与成人表现相似的多系统疾病，常伴有肺门淋巴结肿大和肺部浸润。早发性儿童结节病是该疾病的一种独特形式，其特征为4岁前发病的患者出现皮疹、葡萄膜炎和关节炎三联征。通过活检标本显示典型的非干酪样肉芽肿可确诊结节病。对于多系统受累的儿童结节病，目前的首选治疗方法是使用皮质类固醇。低剂量口服甲氨蝶呤有效且安全，具有激素节省作用。
