■嗜酸性肉芽肿性血管炎(EGPA)是一种罕见的自身免疫性血管炎。IgG4和HBsAg在EGPA中的参与较不常见,但可能发生,并且可能在管理中提出独特的挑战。
■我们提供了一个通过肾活检确诊为EGPA的70岁女性的案例研究。她最初表现为复发性紫癜,腹泻和手脚逐渐麻木,伴随着普遍的软弱。通过一年的醋酸泼尼松和环磷酰胺治疗可实现完全缓解。然而,停止自我药物治疗后,疾病复发,表现为四肢全身皮疹和虚弱。皮肤活检显示嗜酸性粒细胞浸润,炎症细胞主要围绕血管。值得注意的是,治疗期间,患者的乙型肝炎标志物从HBsAg阴性转变为阳性。随后恩替卡韦的管理,随着HBVDNA水平的下降监测,在开始使用类固醇和利妥昔单抗之前再次获得缓解。在分析的其余15名患者中,所有患者均表现出血清IgG4水平升高,没有乙型肝炎检测呈阳性值得注意的是,只有一名患者被诊断为免疫球蛋白G4相关疾病(IgG4-RD),提示单独的IgG4水平升高不一定表明IgG4-RD.
■我们的病例报告强调了EGPA的首次复发,并伴有IgG4升高和乙型肝炎阳性,利妥昔单抗成功治疗。在并发乙型肝炎的情况下,一旦病毒复制得到控制,可以考虑利妥昔单抗治疗。然而,在诱导疾病缓解后,强调维持治疗至关重要。
UNASSIGNED: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of autoimmune vasculitis. The involvement of IgG4 and HBsAg in EGPA is less common but can occur and may present unique challenges in management.
UNASSIGNED: We present a
case study of a 70-year-old female diagnosed with EGPA confirmed via renal biopsy. She initially presented with recurrent purpura, diarrhea and progressive numbness in the hands and feet, accompanied by general weakness. Complete remission was achieved with a one-year course of prednisone acetate and cyclophosphamide treatment. However, upon discontinuation of self-medication, the disease relapsed, manifesting as a generalized rash and weakness in the extremities.Skin biopsy revealed eosinophil infiltration, with inflammatory cells predominantly surrounding blood vessels. Notably, during treatment, the patient\'s hepatitis B markers transitioned from negative to positive for HBsAg. Subsequent administration of entecavir, along with monitoring for a decrease in HBV DNA levels, preceded the initiation of
steroids and rituximab to attain remission once more. Among the remaining 15 patients analyzed, all exhibited elevated serum IgG4 levels, with none testing positive for hepatitis B. Notably, only one patient was diagnosed with immunoglobulin G4-related disease (IgG4-RD), suggesting that elevated IgG4 levels alone may not necessarily indicate IgG4-RD.
UNASSIGNED: Our
case report highlights the first instance of recurrent EGPA accompanied by elevated IgG4 and positivity for hepatitis B, which was successfully treated with rituximab. In cases of concurrent hepatitis B, rituximab treatment may be considered once viral replication is under control. However, emphasis on maintenance therapy is crucial following the induction of disease remission.