Chronic active EBV infection with features of granulomatosis with polyangiitis
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- 作者：Masaki Shimomura ; Hideaki Morishita ; Takaaki Meguro ; Shiro Seto ; Mitsuaki Kimura ; Minoru Hamazaki ; Ayako Hashimoto ; Yuka Sugiyama and Hiroshi Kimura
- 刊名：Pediatrics International
- 出版年：2016
- 出版时间：July 2016
- 年：2016
- 卷：58
- 期：7
- 页码：639-642
- 全文大小：463K
- ISSN：1442-200X
文摘
Herein, we report the case of a 13-year-old boy with multiple recurrent ulcers on his legs. He developed severe sinusitis at 10 years of age and had significant weight loss (6 kg) in the 2 months prior to admission. Histology of tissue biopsied from the ulcer indicated small vessel vasculitis and granulomatous inflammation. Given that these findings met the diagnostic criteria for granulomatosis with polyangiitis (GPA), he was treated with immunosuppressive agents. Further pathology, however, indicated Epstein–Barr virus (EBV)-encoded RNA (EBER) in most lymphocytes in the same sample. The EBER-positive lymphocytes were mainly CD4-positive T cells. The EBV-DNA load in the peripheral blood was also abnormally increased (1.0 × 104 copies/μg DNA). Thus, the diagnosis was established as chronic active EBV infection (CAEBV). This case illustrates the necessity of careful differential diagnosis of CAEBV owing to its clinical resemblance and pathological overlap with GPA.