Prieur A M, Griscelli C
Sem Hop. 1984 Jan 26;60(3):163-7.
The case-reports of seventeen patients who experienced onset of systemic juvenile arthritis between the neonatal period and the age of ten months are studied. Group I consists of six cases in which onset took place after the age of five months. The condition progressed towards recovery with minimal residual joint disease, towards polyarticular involvement or towards perennial systemic manifestations. Group II individualizes three patients with neonatal manifestations, and chronic polymorphonuclear cell meningitis and major radiologic changes as typical features. The eight patients in Group III, some of whom had neonatal onset, exhibited febrile exacerbations with involvement of skin, joints and lymph nodes, often produced by various antigenic stimulations (infections, immunizations, gammaglobulin injections and delayed hypersensitivity reactions). In group III exacerbations were often shorter but more frequent than in group I. After several years follow-up, clinical and radiological evaluation showed no signs of residual joint disease in this group. Biological features in each of the three groups were manifestations of major non-specific inflammation. Histologic examination of lymph nodes and skin showed no specific abnormality. IgA and IgD concentrations were especially high in group III. The reality of the syndrome described many years ago by Wissler and Fanconi appears to be open to debate in the light of manifestations recorded in group III.
对17例在新生儿期至10个月龄之间出现全身型幼年特发性关节炎的患者的病例报告进行了研究。第一组包括6例发病于5个月龄之后的病例。病情发展为恢复，残留关节疾病极少，或发展为多关节受累或出现常年性全身表现。第二组有3例患者表现为新生儿期症状，典型特征为慢性多形核细胞脑膜炎和显著的放射学改变。第三组的8例患者，其中一些发病于新生儿期，表现为发热加重，伴有皮肤、关节和淋巴结受累，常由各种抗原刺激（感染、免疫接种、丙种球蛋白注射和迟发型超敏反应）引起。在第三组中，病情加重通常比第一组持续时间更短但更频繁。经过数年随访，该组的临床和放射学评估未显示残留关节疾病的迹象。三组患者的生物学特征均表现为主要的非特异性炎症。淋巴结和皮肤的组织学检查未发现特异性异常。第三组患者的IgA和IgD浓度尤其高。根据第三组记录的表现，多年前Wissler和Fanconi描述的综合征的真实性似乎有待商榷。
