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继发于细小病毒 B19 感染的全身性红斑狼疮样综合征患者,使用针对肝豆状核变性的螯合剂后,肝衰竭显著改善。

Refractory liver dysfunction was remarkably improved with chelating agents of Wilson's disease, in a patient with systemic lupus erythematosus-like syndrome after a parvovirus B19 infection.

机构信息

Division of Rheumatology and Allergy, Osaka General Medical Center, Osaka, Japan.

Division of Gastroenterology, Osaka General Medical Center, Osaka, Japan.

出版信息

Mod Rheumatol Case Rep. 2021 Jan;5(1):182-187. doi: 10.1080/24725625.2020.1832754. Epub 2020 Oct 23.

DOI:10.1080/24725625.2020.1832754
PMID:33019911
Abstract

Parvovirus B19 infection has been reported to be associated with systemic lupus erythematosus (SLE). Liver dysfunction is frequently observed in SLE patients. However, liver dysfunction caused by an aberrant copper metabolism is rarely seen in patients with parvovirus B19 infection. We report a rare case of SLE-like and Wilson's disease (WD) mimicking symptoms that were simultaneously triggered by parvovirus B19 infection. A 29-year-old man was admitted to our hospital with high-grade fever, arthralgia, and oral ulcers following a parvovirus B19 infection. Laboratory tests showed elevated transaminase levels, proteinuria, anti-double-stranded DNA antibody, high levels of serum ferritin, and leukocytopenia. He was suspected of having SLE with haemophagocytosis and was treated with high doses of prednisolone. Subsequently, the patient's arthritis symptoms improved and the proteinuria improved. Immunosuppressive therapies improved most of his symptoms except for the high titre of transaminases were alleviated. Laboratory findings indicated low serum levels of ceruloplasmin and copper along with elevated levels of 24-hour urinary copper. Liver biopsy detected copper in hepatocytes. Although the hepatic copper content was relatively low in this case, the dysregulation of copper metabolism was considered to be a main cause of his elevated levels of liver enzymes. Therefore, we started treatment with chelating agents used in WD treatment. At the 2-month follow-up, the liver dysfunction had significantly improved. Our case suggests that in patients with refractory liver dysfunction due to unknown reasons, it is necessary to exclude the possibility that an abnormal copper metabolism had caused an increase in the levels of liver enzymes.

摘要

微小病毒 B19 感染与系统性红斑狼疮(SLE)有关。肝功能障碍在 SLE 患者中很常见。然而,微小病毒 B19 感染引起的铜代谢异常导致的肝损伤很少见。我们报告了一例罕见的微小病毒 B19 感染同时引发类似系统性红斑狼疮和威尔逊病(WD)表现的病例。一名 29 岁男性因微小病毒 B19 感染后出现高热、关节炎和口腔溃疡而入院。实验室检查显示转氨酶水平升高、蛋白尿、抗双链 DNA 抗体、血清铁蛋白水平升高和白细胞减少。他被怀疑患有噬血细胞性系统性红斑狼疮,并接受了大剂量泼尼松龙治疗。随后,患者的关节炎症状改善,蛋白尿改善。除了转氨酶水平仍然较高外,免疫抑制治疗改善了他的大部分症状。实验室检查结果表明血清铜蓝蛋白和铜水平降低,24 小时尿铜水平升高。肝脏活检显示肝细胞中有铜。尽管本例肝脏铜含量相对较低,但铜代谢失调被认为是导致肝酶升高的主要原因。因此,我们开始使用 WD 治疗中使用的螯合剂进行治疗。在 2 个月的随访中,肝功能显著改善。我们的病例表明,对于不明原因导致的难治性肝功能障碍患者,有必要排除异常铜代谢导致肝酶水平升高的可能性。

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