Takei S, Maeno N, Shigemori M, Nakae Y, Mori H, Nerome Y, Imanaka H, Hokonohara M, Miyata K
Department of Pediatrics, Faculty of Medicine, Kagoshima University, Kagoshima-city.
Ryumachi. 1997 Oct;37(5):702-8.
In order to discuss the diversity of clinical features and the difficulty in diagnosis of children with juvenile rheumatoid arthritis (JRA), we present two cases who have documented the development of systemic lupus erythematosus (SLE) and mixed connective tissue disease (MCTD) after a long period of disease characterized only by arthritis that was initially diagnosed as JRA. The first case was a girl diagnosed for her arthritic joints as polyarticular JRA at 15 years of age. At onset, she had Raynaud phenomenon but autoantibodies such as anti-nuclear antibody (ANA), anti-DNA antibody, and rheumatoid factor were negative. Five years after onset, she became ANA positive and 3 years later she became pregnant. During her pregnancy, she became positive for anti-DNA antibody without any signs of nephritis. One month after the delivery, however, she developed butterfly rash, carditis, nephritis, and was diagnosed as SLE. No destructive changes were observed in her joints though arthritis continued for 8 years form onset to pregnancy. The second case was a 3 years old girl who was diagnosed as polyarticular JRA. Treatment by aspirin induced complate remission after one year from the onset. However, 10 years after that remission, she developed Raynaud phenomenon and arthralgia in her knees and hip joints. Her laboratory findings showed hypergammaglobulinemia, positive ANA, positive anti-DNA antibody, positive anti-RNP antibody. She was eventually diagnosed as MCTD when she was found to have polymyositis by EMG and serum CK. In the present paper, two cases imply the difficulty in diagnosing JRA and diversity of rheumatic diseases such as JRA, SLE and MCTD. Closer and longer period of observation is essential for the JRA patients with nondestructive arthritis.
为了探讨青少年类风湿关节炎（JRA）患儿临床特征的多样性及诊断困难，我们报告两例病例，这两例患儿最初仅以关节炎为特征，最初被诊断为JRA，经过长时间病程后分别发展为系统性红斑狼疮（SLE）和混合性结缔组织病（MCTD）。第一例是一名15岁女孩，因关节炎症被诊断为多关节型JRA。起病时，她有雷诺现象，但抗核抗体（ANA）、抗DNA抗体和类风湿因子等自身抗体均为阴性。起病5年后，她ANA转为阳性，3年后怀孕。孕期，她抗DNA抗体转为阳性，但无肾炎迹象。然而，分娩后1个月，她出现蝶形红斑、心肌炎、肾炎，被诊断为SLE。尽管从起病到怀孕关节炎持续了8年，但关节未观察到破坏性改变。第二例是一名3岁女孩，被诊断为多关节型JRA。阿司匹林治疗1年后病情完全缓解。然而，缓解10年后，她出现雷诺现象，膝关节和髋关节疼痛。实验室检查发现高球蛋白血症、ANA阳性、抗DNA抗体阳性、抗RNP抗体阳性。当通过肌电图和血清肌酸激酶发现她患有多发性肌炎时，最终被诊断为MCTD。在本文中，两例病例提示JRA诊断困难以及JRA、SLE和MCTD等风湿性疾病的多样性。对于无破坏性关节炎的JRA患者，密切和长期观察至关重要。
