Häfner R, Vogel P
Children's Hospital, Department of Pediatric Rheumatology, Garmisch-Partenkirchen, Germany.
Clin Exp Rheumatol. 1993 Nov-Dec;11(6):685-91.
The clinical picture of twelve patients with sarcoidosis commencing under 4 years of age is presented. Follow-up ranged from 5 to 30 years with a mean of 11.5 years. Two patients were mother and daughter, the other cases were sporadic. All children developed the typical symptom triad of rash, polyarthritis and uveitis with onset before their 4th birthday. Further symptoms comprised fever, hepatosplenomegaly, parotid swelling and hypertension. Eight patients suffered from cardiac and four from cerebral involvement; one girl died from cerebral involvement at the age of 12. Two developed severe lung changes. The prognosis was poor with sequelae in all patients. Several children seem to have improved on immunosuppressive therapy. Early onset sarcoidosis is a multisystemic granulomatous disease; since polyarthritis is a dominant manifestation, many patients are diagnosed as having juvenile chronic arthritis. The early onset form must be distinguished from later onset sarcoidosis, which mainly affects children over 8 years of age or young adults. The disease types differ in their presentation, symptoms, course and prognosis; some of the clinical features and the histology, however, are identical.
本文呈现了12例4岁以下起病的结节病患儿的临床情况。随访时间为5至30年，平均11.5年。其中2例为母女，其他病例为散发病例。所有患儿均在4岁生日前出现典型的皮疹、多关节炎和葡萄膜炎三联征。其他症状包括发热、肝脾肿大、腮腺肿大和高血压。8例患儿有心脏受累，4例有脑部受累；1名女孩12岁时死于脑部受累。2例出现严重肺部病变。所有患者预后均较差并伴有后遗症。部分患儿接受免疫抑制治疗后病情似乎有所改善。早发型结节病是一种多系统肉芽肿性疾病；由于多关节炎是主要表现，许多患者被诊断为幼年慢性关节炎。早发型结节病必须与晚发型结节病相鉴别，后者主要影响8岁以上儿童或年轻成人。这两种疾病类型在表现、症状、病程和预后方面有所不同；然而，某些临床特征和组织学表现是相同的。
