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韦格纳肉芽肿病的肝脏受累:一例报告。

Hepatic involvement in Wegener's granulomatosis: a case report.

作者信息

Goritsas Constantin, Paissios Nicolas P, Trigidou Rodoula, Delladetsima Joanna

机构信息

Department of Internal Medicine, Sotiria General Hospital, 152 Mesogeion Avenue, Athens, 11527, Greece.

出版信息

J Med Case Rep. 2010 Jan 14;4:9. doi: 10.1186/1752-1947-4-9.

Abstract

INTRODUCTION

We report the case of a 58-year-old Caucasian Greek man who presented with dry cough, fever, bilateral alveolar infiltrates and acute hepatitis.

CASE PRESENTATION

After a lung biopsy, the patient was diagnosed with Wegener's granulomatosis. The diagnosis was supported by the presence of anti-proteinase-3 anti-neutrophil cytoplasmic antibodies. A liver biopsy demonstrated the presence of mild non-specific lobular hepatitis and periodic acid-Schiff positive Lafora-like inclusions in a large number of his liver cells. The patient was treated with prednisone and cyclophosphamide, which was followed by subsequent remissions of chest X-ray findings and liver function studies.

CONCLUSION

What makes this case worth reporting is the coexistence of liver inflammation with a biochemical profile of severe anicteric non-viral, non-drug induced hepatitis coinciding with the diagnosis of Wegener's granulomatosis. Our paper may be the first report of hepatic involvement in a patient diagnosed with Wegener's granulomatosis. The aetiological link between the two diseases is supported by the reversion of hepatitis after the immunosuppression of Wegener's granulomatosis. We favor the hypothesis that hepatic vasculitis may be the cause of acute hepatocellular necrosis.

摘要

引言

我们报告一例58岁的希腊裔白种男性病例,该患者出现干咳、发热、双侧肺泡浸润及急性肝炎。

病例介绍

经肺活检后,该患者被诊断为韦格纳肉芽肿。抗蛋白酶-3抗中性粒细胞胞浆抗体的存在支持了这一诊断。肝活检显示存在轻度非特异性小叶性肝炎,且其大量肝细胞中有过碘酸希夫染色阳性的拉福拉样包涵体。患者接受泼尼松和环磷酰胺治疗,随后胸部X光检查结果和肝功能研究出现缓解。

结论

该病例值得报告之处在于,在诊断韦格纳肉芽肿的同时,出现了伴有严重无黄疸型非病毒性、非药物性肝炎生化特征的肝脏炎症。我们的论文可能是关于韦格纳肉芽肿患者肝脏受累的首例报告。韦格纳肉芽肿免疫抑制治疗后肝炎症状的缓解支持了这两种疾病之间的病因学联系。我们支持肝血管炎可能是急性肝细胞坏死病因的假说。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1046/2821396/9d71ad66c721/1752-1947-4-9-1.jpg

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