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An overlap syndrome involving autoimmune hepatitis and systemic lupus erythematosus in childhood

机译:儿童自身免疫性肝炎和系统性红斑狼疮的重叠综合征

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摘要

We report a 12 years old female patient with an overlap syndrome involving autoimmune hepatitis (AIH) and systemic lupus erythematosus (SLE). The patient presented with jaundice, hepatosplenomegaly, malaise, polyarthralgia, arthritis and butterfly rash on the face. Laboratory tests revealed severe liver dysfunction, Coombs positive hemolytic anemia and a positive ANA/ anti-dsDNA test. Renal biopsy showed class IIA kidney disease, while liver biopsy showed chronic hepatitis with severe inflammatory activity. The patient satisfied the international criteria for both SLE and AIH. Clinical symptoms and laboratory findings of SLE improved with high dose treatment with corticosteroids and azathioprine, however, remission of the liver disease could not be achieved. Repeat biopsy of the liver after three years of therapy revealed ongoing chronic hepatitis with high level of inflammatory activity. The present case indicates that children with liver dysfunction and SLE should be investigated for AIH. There is much diagnostic and therapeutic dilemma in patients with AIH-SLE overlap syndrome.
机译:我们报告了一名患有自身免疫性肝炎(AIH)和系统性红斑狼疮(SLE)的重叠综合征的12岁女性患者。患者出现黄疸,肝脾肿大,全身不适,多关节痛,关节炎和蝴蝶斑疹。实验室检查显示严重肝功能不全,库姆斯阳性溶血性贫血和ANA /抗dsDNA检测阳性。肾活检显示为IIA级肾脏疾病,而肝活检显示为慢性肝炎,具有严重的炎症活动。患者符合SLE和AIH的国际标准。大剂量皮质类固醇和硫唑嘌呤治疗可改善SLE的临床症状和实验室检查结果,但是,肝病无法缓解。治疗三年后,重复进行肝脏活检,发现正在进行的慢性肝炎具有较高的炎症活性。目前的情况表明,应该对患有肝功能不全和SLE的儿童进行AIH检查。 AIH-SLE重叠综合征患者存在许多诊断和治疗难题。

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