Yamashita H, Shimizu A, Tsuchiya H, Takahashi Y, Kaneko H, Kano T, Mimori A
Division of Rheumatic Diseases, National Center for Global Health and Medicine, Shinjuku-ku, Tokyo, Japan
Division of Rheumatic Diseases, National Center for Global Health and Medicine, Shinjuku-ku, Tokyo, Japan.
Lupus. 2014 Jul;23(8):833-6. doi: 10.1177/0961203314527367. Epub 2014 Mar 7.
Chronic active Epstein-Barr virus infection (CAEBV) is characterized by chronic infectious mononucleosis-like symptoms. We report a very rare case with autoimmune hepatitis (AIH) complicated by CAEBV. A 50-year-old woman with systemic lupus erythematosus (SLE) complicated by AIH began to suffer from acute respiratory failure and her clinical symptoms improved rapidly in response to steroid treatment. However, during the gradual tapering of the steroid dose, a steady increase of the serum hepatobiliary enzyme levels subsequently was observed and the patient began to have continuous fever. Moreover, upper gastrointestinal endoscopy revealed multiple intractable gastric ulcers. When EBER-ISH was performed on liver biopsy and gastric mucosal biopsy specimens, EBER-positive lymphocytes were observed. When peripheral blood was examined, 2.1 × 10(6) copies/µg of EBV-DNA were observed in the CD4-positive T cells, confirming the diagnosis of CAEBV. A cooling therapy was started by steroid and cyclosporine. Thereafter, despite the start of CHOP therapy, she developed a malignant lymphoma (PTCL-NOS) and died of hepatic failure. When treatment-resistant AIH patients are encountered, not only AIH exacerbation but also CAEBV should be considered in the differential diagnosis.
慢性活动性EB病毒感染（CAEBV）的特征为慢性传染性单核细胞增多症样症状。我们报告了1例极为罕见的自身免疫性肝炎（AIH）合并CAEBV的病例。1名50岁女性，患有系统性红斑狼疮（SLE）并合并AIH，开始出现急性呼吸衰竭，经类固醇治疗后临床症状迅速改善。然而，在逐渐减少类固醇剂量的过程中，随后观察到血清肝胆酶水平持续升高，且患者开始持续发热。此外，上消化道内镜检查发现多处难治性胃溃疡。对肝活检和胃黏膜活检标本进行EBER原位杂交（EBER-ISH）时，观察到EBER阳性淋巴细胞。检查外周血时，在CD4阳性T细胞中检测到2.1×10(6)拷贝/μg的EBV-DNA，确诊为CAEBV。开始使用类固醇和环孢素进行降温治疗。此后，尽管开始了CHOP治疗，但她仍发展为恶性淋巴瘤（非特指外周T细胞淋巴瘤），并死于肝衰竭。当遇到治疗抵抗的AIH患者时，鉴别诊断中不仅应考虑AIH病情加重，还应考虑CAEBV。
