腹腔积液1月余,肾功能异常2周

Ascites for more than One Month,Renal Insufficiency for Two Weeks

Castleman病和POEMS综合征属于罕见病,二者关系密切,但临床对其肾脏损伤认识不多。本例患者为44岁男性,病初表现为顽固性腹腔积液,之后出现肾功能异常及下肢水肿,查体发现颈胸部多发血管瘤,炎症指标及血清血管内皮生长因子明显升高,M蛋白阴性。皮肤活检示肾小球样血管瘤,肾活检表现为内皮细胞病及小管间质损害,淋巴结活检诊断为Castleman病。加用足量泼尼松和环磷酰胺后,病情迅速缓解,肾功能恢复正常。

Castleman disease and POEMS syndrome are both rare diseases,and there is a close relationship between each other. Although these two diseases have been investigated for decades,a comprehensive understanding of renal manifestations is still lacking. We reported a 44-year-old man who suffered from refractory ascites initially, and then developed renal insufficiency and lower limb edema. Physical examination at admission indicated multiple hemangioma in the zone of neck and chest. There was a significant elevation in inflammatory indices and serum vas-cular endothelial growth factor,while the monoclonal protein was negative. The lymph node biopsy showed Castle-man disease; and the skin biopsy showed glomeruloid hemangioma. Renal biopsy demonstrated endotheliopathy and tubular-interstitial injury. He was diagnosed with Castleman disease; and after the treatment with high dose glu-cocorticoid and immunosuppressants,the disease had been relieved rapidly and the renal function returned normal.【