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

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