幼年型慢性关节炎伴发葡萄膜炎患者的临床特征和诊断及治疗

The clinical feature, diagnosis and treatment of uveitis associated with juvenile chronic arthritis

目的 探讨幼年型慢性关节炎(JCA)伴发葡萄膜炎的临床特征、诊断及治疗。方法对1996至2002年间于中山大学中山眼科中心葡萄膜炎专科就诊的26例JCA伴发葡萄膜炎患者采集病史,进行常规裂隙灯显微镜、检眼镜、抗核抗体(ANA)、红细胞沉降率(ESR)、类风湿因子(RF)、C反应蛋白(CRP)及抗链球菌溶血素“O”(ASO)等检查。根据临床需要,部分患者进行人类白细胞相关抗原B27(HLA B27)检测和骶髂关节及脊柱X线检查,并分析患者的治疗、预后及并发症等情况。结果 26例患者中男性11例,女性15例;JCA和葡萄膜炎的中位发病年龄分别为(8±7)岁和(9±5)岁;其中慢性前葡萄膜炎21例,急性前葡萄膜炎3例,慢性全葡萄膜炎2例; 22例患者双眼患病, 4例单眼患病,患眼共有48只;其中33只眼有轻至中度前房闪辉, 24只眼有少至中等量房水细胞, 39只眼发生虹膜后粘连; 30只眼并发白内障, 20只眼并发角膜带状变性, 12只眼继发青光眼。实验室检查发现18例患者呈ANA阳性,ASO均为阴性,RF仅1例为阳性。炎性反应急性期给予睫状肌麻痹剂、糖皮质激素滴眼治疗。病情较顽固的6例患者中, 3例加用环孢霉素A口服治疗, 3例加用苯丁酸氮芥口服治疗,炎性反应均良好控制。16只眼由于治疗前已合并严重的并发症,视力无显著提高;余32只眼视力均有不同程度提高。

Objective To investigate the clinical features, diagnosis and treatment of uveitis associated with juvenile chronic arthritis (JCA). Methods A retrospective study was performed on the clinical data of 26 patients with uveitis associated with JCA, referred to Zhongshan Ophthalmic Center from 1996 to 2002. Taking of history, examination with slit-lamp microscope and ophthalmoscope were carefully performed in all of these patients. Laboratory tests including antinuclear antibodies, erythrocyte sedimentation rate, rheumatoid factor, C reactive protein and antistreptolysin O were used to disclose possible causes. Human leukocyte antigen B 27 and X-ray examination of sacroiliac joints and spine were carried out if necessary. Data about the treatment, visual outcome and complications were analyzed . Results Twenty-six patients, 11 males and 15 females, were included in the present studies. Age of onset of JCA and uveitis averaged 8 years and 9 years, respectively. Twenty-one patients had chronic anterior uveitis. Acute anterior uveitis and chronic panuveitis were noted in 3 and 2 patients, respectively. Twenty-two patients had bilateral uveitis, the other 4 had unilateral involvement. The ophthalmologic examination revealed that 33 of the 48 affected eyes showed mild aqueous humor flare, 24 had few cells in the anterior chamber. Complicated cataract, band keratopathy and secondary glaucoma were noted in 30, 20 and 12 eyes, respectively. The laboratory examination revealed positive antinuclear antibodies in 18 cases and rheumatoid factor positive in one case. In acute episode, patients were treated with extensive topical cycloplegic agents and corticosteroids eyedrops. In the 6 cases with severe uveitis, 3 patients were treated with cyclosporine A (5 mg·kg -1 ·d -1 )and the other 3 were treated with chlorambucil (0.1 mg·kg -1 ·d -1 ). The intraocular inflammation in all of these patients was satisfactorily controlled with these treatments. Visual acuity improvement was noted in 32 affected eyes but not in the other 16 eyes which had already serious complications before the treatment. Conclusions Uveitis associated with JCA is characterized by a chronic and recurred iridocyclitis, which usually developed within 5 year after JCA onset. Although the intraocular inflammation associated with JCA is usually white uveitis, complications such as cataract, secondary glaucoma and keratopathy occur frequently in these patients. The diagnosis is mainly based on typical clinical manifestations, the history of arthritis and positive antinuclear antibodies. Adequate application of cycloplegic agents, corticosteroids eyedrops, immunosupressives and the regular follow-up should be kept in mind in the treatment of these patients.