Unsal Erbil, Arli Ayse Ozgun, Akman Hakki
Dokuz Eylul University, Faculty of Medicine, Department of Pediatrics, Division of Immunology-Rheumatology, Balcova 35340 Izmir, Turkiye.
Pediatr Rheumatol Online J. 2007 May 4;5:7. doi: 10.1186/1546-0096-5-7.
An 8.5-year-old girl was referred with swelling of both knees lasting for two years. ANA was found as negative. She was diagnosed as oligoarticular JIA. After two years of follow-up, thrombocytopenia was detected during routine screening. Her ANA and anti ds-DNA antibodies also became positive, with low levels of C3 and C4. She was diagnosed as Juvenile SLE, meeting the criteria cytopenia, positive immunoserology (anti dsDNA), positive ANA test, and four years of ongoing chronic arthritis, so called as "rhupus arthropathy". We should be aware of the several initial incomplete presentations of lupus in children. We should be careful in monitoring the serious manifestations of the disease in juvenile lupus patients with rhupus arthropathy, and consider the poor response to standard disease modifying agents.
一名8.5岁女童因双膝肿胀两年前来就诊。抗核抗体(ANA)检测为阴性。她被诊断为少关节型幼年特发性关节炎(JIA)。经过两年的随访,在常规筛查中发现血小板减少。她的抗核抗体和抗双链DNA抗体也呈阳性,补体C3和C4水平较低。她被诊断为青少年系统性红斑狼疮(SLE),符合血细胞减少、免疫血清学阳性(抗双链DNA)、抗核抗体检测阳性以及持续四年的慢性关节炎等标准,即所谓的“rhupus关节炎”。我们应该意识到儿童狼疮最初的几种不完整表现。对于患有rhupus关节炎的青少年狼疮患者,我们应谨慎监测疾病的严重表现,并考虑其对标准疾病改善药物反应不佳的情况。