[ 摘要 ] 1 例 24 岁男性患者具急性肾炎综合征、肝功能损害、血免疫学异常合并肠系膜巨大包块，经肾穿刺和肠系膜包块活检确诊为“狼疮性肾炎Ⅴ + Ⅲ型”，合并 Castleman 病，给予激素和环磷酰胺诱导治疗后联合应用利妥昔单抗。随访第 3 个月狼疮性肾炎部分缓解，系统性红斑狼疮活动度评分（SLEDAI）由 20 分（重度活动）下降至 4 分（基本不活动）。随访至第 9 个月狼疮性肾炎临床完全缓解，肠系膜包块缩小 50%。狼疮性肾炎合并多中心型 Castleman 病，必须依靠病理学诊断，极易漏诊和误诊，传统免疫抑制剂治疗预后差，应用激素和环磷酰胺诱导治疗后，联合靶向治疗药物利妥昔单抗，可获得较好疗效。

[Abstract] A 24-year-old male suffered from acute nephritic syndrome, liver dysfunction, and mesenteric mass. Laboratory examination showed a variety
of autoantibodies (ANA, SM, and A-β2-GP1) were positive. The biopsies of the kidney and the mesenteric mass were performed. The diagnosis was type
Ⅴ + Ⅲ lupus nephritis accompanied with Castleman’s disease. Then the patient was given induction therapy of glucocorticoids and cyclophosphamide for
the first 3 months, followed by rituximab as maintenance therapy. The patient was followed up after 0, 3, and 9 months. After 3-month treatment, lupus
nephritis was partially remitted, and systemic lupus erythematosus disease activity index (SLEDAI) decreased to 4 scores in an inactivity phase from 20
scores in a serious activity phase at baseline. Nine months later, lupus nephritis was completely remitted and 50% mesenteric mass was regressed through CT scanning. Lupus nephritis can accompany with multicentric Castleman’s disease. Due to lack of clinical specificity and effective therapy, patients may have a high misdiagnosis rate and poor prognosis. The most reliable way to establish a definitive diagnosis relays on histopathologic confirmation.
The management of induction therapy of glucocorticoids and cyclophosphamide, followed maintenance therapy of rituximab may become a beneficial
treatment.