Pediatric Rheumatology Unit, Children's Hospital, São Paulo, Brazil.
Lupus. 2011 Aug;20(9):960-4. doi: 10.1177/0961203311400113. Epub 2011 Jun 2.
To evaluate the prevalence of chronic polyarthritis in juvenile systemic lupus erythematosus (JSLE) and to describe the manifestations, treatments, and outcomes in these patients.
From January 1983 to July 2010, 5419 patients were followed up at the Pediatric Rheumatology Unit of the University Hospital and 271 (5%) of them had JSLE (American College of Rheumatology [ACR] criteria). 'Rhupus' was classified as the overlap of juvenile idiopathic arthritis (International League of Associations for Rheumatology [ILAR] criteria) and JSLE. We evaluated demographic data, polyarthritis and other clinical manifestations, disease activity and damage, laboratory exams, radiographic findings, treatments, and outcomes.
The prevalence of chronic polyarthritis in this JSLE population was 2.6% (7/271). This articular involvement was the initial manifestation in all seven JSLE patients. The median duration of chronic polyarthritis was 11 months (range 2-15 months). Interestingly, rhupus with chronic polyarthritis and limitation of movement, presence of rheumatoid factor, autoantibodies, and/or radiographic abnormalities (juxtaarticular osteopenia, joint-space narrowing, or erosions) was evidenced in three patients. No patient had deformities of hands and feet associated with Jaccoud's arthropathy or osteonecrosis. All patients were treated with nonsteroidal anti-inflammatory drugs (NSAIDs, naproxen 10-15 mg/kg/day) when polyarthritis diagnosis was established. Prednisone and antimalarials were administered at JSLE diagnosis. The three non-responsive rhupus patients were treated in conjunction with immunosuppressive drugs (methotrexate, azathioprine, and/or cyclosporine).
Chronic polyarthritis was a rare lupus manifestation in active pediatric patients. The interesting overlap between chronic arthritis and lupus, called rhupus suggests a new entity with a different clinical profile and a poor response to treatment with NSAIDs alone. In addition, the occurrence of this association in JSLE patients could be classified as a clinical sub-group of JSLE with possible specific genetic determinants.
评估幼年系统性红斑狼疮（JSLE）中慢性多发性关节炎的患病率，并描述这些患者的表现、治疗和结局。
1983 年 1 月至 2010 年 7 月，5419 例患者在大学医院儿科风湿病科接受随访，其中 271 例（5%）患有 JSLE（美国风湿病学会[ACR]标准）。“Rhupus”被归类为幼年特发性关节炎（国际风湿病联盟[ILAR]标准）和 JSLE 的重叠。我们评估了人口统计学数据、多发性关节炎和其他临床表现、疾病活动和损伤、实验室检查、影像学发现、治疗和结局。
该 JSLE 人群中慢性多发性关节炎的患病率为 2.6%（7/271）。所有 7 例 JSLE 患者均以关节受累为首发表现。慢性多发性关节炎的中位持续时间为 11 个月（范围 2-15 个月）。有趣的是，3 例 rhupus 患者存在慢性多发性关节炎和运动受限、类风湿因子、自身抗体和/或影像学异常（关节旁骨质疏松、关节间隙变窄或侵蚀）。无患者存在与 Jac coud 关节炎或骨坏死相关的手脚畸形。所有患者在诊断为多发性关节炎时均接受非甾体抗炎药（NSAIDs，萘普生 10-15mg/kg/天）治疗。在诊断为 JSLE 时给予泼尼松和抗疟药。3 例对 rhupus 反应不佳的患者联合使用免疫抑制剂（甲氨蝶呤、硫唑嘌呤和/或环孢素）治疗。
慢性多发性关节炎是活动性儿科患者中罕见的狼疮表现。慢性关节炎和狼疮之间有趣的重叠，称为 rhupus，提示存在一种新的实体，其临床特征不同，单独使用 NSAIDs 治疗反应不佳。此外，JSLE 患者中发生这种关联可归类为 JSLE 的一个临床亚组，可能存在特定的遗传决定因素。
