Duzova Ali, Bakkaloglu Aysin
Department of Pediatrics, Pediatric Nephrology and Rheumatology Unit, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Curr Pharm Des. 2008;14(13):1295-301. doi: 10.2174/138161208799316339.
Central nervous system (CNS) manifestations are not rare in pediatric rheumatic diseases. They may be a relatively common feature of the disease, as in systemic lupus erythematosus (SLE) and Behçet's disease. Direct CNS involvement of a systemic rheumatic disease, primary CNS vasculitis, indirect involvement secondary to hypertension, hypoxia and metabolic changes, and drug associated adverse events may all result in CNS involvement. We have reviewed the CNS manifestations of SLE, Behçet's disease, Henoch-Schönlein purpura, polyarteritis nodosa, juvenile idiopathic arthritis, juvenile ankylosing spondylitis, familial Mediterranean fever, scleroderma, sarcoidosis, Wegener's granulomatosis, Takayasu's arteritis, CINCA syndrome, Kawasaki disease, and primary CNS vasculitis; and adverse CNS effects of anti-rheumatic drugs in pediatric patients. The manifestations are diverse; ranging from headache, seizures, chorea, changes in personality, depression, memory and concentration problems, cognitive impairment, cerebrovascular accidents to coma, and death. The value of cerebrospinal fluid (CSF) examination (pleocytosis, high level of protein), auto-antibodies in serum and CSF, electroencephalography, neuroimaging with computerized tomography, magnetic resonance imaging, SPECT, PET, and angiography depends on the disease. Brain biopsy is gold standard for the diagnosis of CNS vasculitis, however it may be inconclusive in 25% of cases. A thorough knowledge of the rheumatic diseases and therapy-related adverse events is mandatory for the management of a patient with rheumatic disease and CNS involvement. Severe CNS involvement is associated with poor prognosis, and high mortality rate. High dose steroid and cyclophosphamide (oral or intravenous) are first choice drugs in the treatment; plasmapheresis, IVIG, thalidomide, and intratechal treatment may be valuable in treatment-resistant, and serious cases.
中枢神经系统（CNS）表现在儿科风湿性疾病中并不罕见。它们可能是疾病的一个相对常见的特征，如在系统性红斑狼疮（SLE）和白塞病中。系统性风湿性疾病直接累及中枢神经系统、原发性中枢神经系统血管炎、继发于高血压、缺氧和代谢变化的间接累及以及药物相关不良事件均可能导致中枢神经系统受累。我们回顾了SLE、白塞病、过敏性紫癜、结节性多动脉炎、幼年特发性关节炎、幼年强直性脊柱炎、家族性地中海热、硬皮病、结节病、韦格纳肉芽肿、大动脉炎、慢性婴儿神经皮肤关节综合征（CINCA综合征）、川崎病和原发性中枢神经系统血管炎的中枢神经系统表现；以及儿科患者抗风湿药物的中枢神经系统不良影响。其表现多种多样；包括头痛、癫痫发作、舞蹈病、性格改变、抑郁、记忆和注意力问题、认知障碍、脑血管意外直至昏迷和死亡。脑脊液（CSF）检查（细胞增多、蛋白水平升高）、血清和脑脊液中的自身抗体、脑电图、计算机断层扫描神经成像、磁共振成像、单光子发射计算机断层扫描（SPECT）、正电子发射断层扫描（PET）和血管造影的价值取决于疾病。脑活检是诊断中枢神经系统血管炎的金标准，然而在25%的病例中可能无法得出结论。对于患有风湿性疾病并累及中枢神经系统的患者的管理，必须全面了解风湿性疾病和治疗相关不良事件。严重的中枢神经系统受累与预后不良和高死亡率相关。高剂量类固醇和环磷酰胺（口服或静脉注射）是治疗的首选药物；血浆置换、静脉注射免疫球蛋白（IVIG）、沙利度胺和鞘内治疗可能对治疗抵抗的严重病例有价值。
