上海交通大学学报（医学版）

上海交通大学学报（医学版） ›› 2017, Vol. 37 ›› Issue (12): 1710-.doi: 10.3969/j.issn.1674-8115.2017.12.026

• 病例报告 • 上一篇    

狼疮性肾炎合并 Castleman 病 1 例报道及文献复习

赵初娴 1，高 峰 2，戎 殳 3，尚明花 3   

  1. 上海交通大学附属第一人民医院 1. 血液科，2. 病理科，3. 肾内科，上海 200080
  • 出版日期:2017-12-28 发布日期:2018-01-10
  • 通讯作者: ?尚明花，电子信箱：shangminghua@medmail.com.cn。
  • 作者简介:赵初娴（1976—），女，主治医师，硕士；电子信箱：zhaochuxian@medmail.com.cn。

Lupus nephritis accompanied with Castleman’s disease: a case report and literature review

ZHAO Chu-xian1, GAO Feng2, RONG Shu3, SHANG Ming-hua3#br#   

  1. 1. Department of Hematology, 2. Department of Pathology, 3.Department of Nephrology, Shanghai General Hospital, Shanghai Jiao Tong University, Shanghai 200080, China
  • Online:2017-12-28 Published:2018-01-10

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摘要： [ 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。

关键词: &ensp, Castleman 病；狼疮性肾炎；自身免疫性疾病；利妥昔单抗

Abstract:

[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.

Key words: Castleman’s disease, lupus nephritis, autoimmune disease, rituximab