Deen M E J, Porta G, Fiorot F J, Campos L M A, Sallum A M E, Silva C A A
Paediatric Rheumatology, Division of Rheumatology and Hepatology Units of Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
Lupus. 2009 Jul;18(8):747-51. doi: 10.1177/0961203308100559.
Juvenile systemic lupus erythematosus (JSLE) and autoimmune hepatitis (AIH) are both autoimmune disorders that are rare in children and have a widespread clinical manifestation. A few case reports have shown a JSLE-AIH associated disorder. To our knowledge, this is the first study that simultaneously evaluated the prevalence of JSLE-AIH in a large JLSE and AIH population in groups of Hepatology and Rheumatology of a tertiary Paediatric University Hospital. In a 24-year period, 228 patients were diagnosed with JSLE (ACR criteria). In the same period, 252 patients were diagnosed with AIH according to the International Autoimmune Hepatitis Group. In this article, we present the demographic data, clinical features, laboratory exams and treatment of four children with both the diseases. The prevalence was 1.8% in JSLE population and was 1.6% in AIH population. The current median age was 15.5 years and three were females. In three of them, the diagnosis of AIH preceded JSLE. All of them had increased liver enzymes with a characteristic liver biopsy of AIH and responded to the combination of prednisone, azathioprine and antimalarial drugs. In conclusion, the presence of AIH-JSLE associated disorder was rarely observed. The liver biopsy could be necessary in patients with JLSE with a persistent increase of liver enzymes.
青少年系统性红斑狼疮（JSLE）和自身免疫性肝炎（AIH）均为自身免疫性疾病，在儿童中较为罕见，且临床表现广泛。少数病例报告显示存在JSLE-AIH相关疾病。据我们所知，这是第一项在一家三级儿科大学医院的肝病科和风湿病科的大量JLSE和AIH患者群体中同时评估JSLE-AIH患病率的研究。在24年期间，228例患者被诊断为JSLE（美国风湿病学会标准）。同期，根据国际自身免疫性肝炎小组标准，252例患者被诊断为AIH。在本文中，我们介绍了4例同时患有这两种疾病的儿童的人口统计学数据、临床特征、实验室检查和治疗情况。JSLE患者群体中的患病率为1.8%，AIH患者群体中的患病率为1.6%。当前的中位年龄为15.5岁，3例为女性。其中3例中，AIH的诊断先于JSLE。他们均有肝酶升高，伴有AIH特征性肝活检表现，并对泼尼松、硫唑嘌呤和抗疟药物联合治疗有反应。总之，很少观察到AIH-JSLE相关疾病。对于肝酶持续升高的JLSE患者，可能需要进行肝活检。
