狼疮性肾炎合并Castleman病1例报道及文献复习

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

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。

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 typeⅤ ＋ 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.