POEMS综合征伴弥漫性增生性肾小球肾炎1例并文献复习

POEMS syndrome combined with diffuse proliferative glomerulonephritis:A case report and literature review

目的报道POEMS综合征(polyneuropathy,organomegaly,endocrinopathy,M protein elevation,and skin changes syndrome)伴弥漫性增生性肾小球肾炎1例,提高临床医师对POEMS综合征系统损害的认识。方法对华中科技大学同济医学院附属梨园医院2018年10月2日收治的1例合并弥漫性增生性肾小球肾炎的POEMS综合征进行回顾并复习文献。结果患者男,70岁,以水肿、高血压为首发症状,同时合并多发淋巴结肿大、多发性骨硬化、多浆膜腔积液、蛋白尿及IGA-KAP+IGG-LAM双M蛋白血症。肾病理示:弥漫性增生性肾小球肾炎;淋巴结活检示:血管滤泡性淋巴结增生(Castleman病)透明血管型。初始拟诊为Castleman病合并弥漫性增生性肾小球肾炎,使用沙利度胺治疗后,患者症状改善不明显,尿蛋白持续存在,同时新发心包积液,双下肢乏力。复习文献,考虑POEMS综合征可能,行血管内皮生长因子检测:1 423.08 pg/ml↑,肌电图检查示周围神经病变,证实了该诊断。予以RCOP(利妥昔单抗+环磷酰胺+长春瑞滨+泼尼松)化疗半年后症状明显改善,血管内皮生长因子水平下降。结论 POEMS综合征肾损害少见,且更易发生在合并Castleman病的患者中,因此两者并存时,不应忽视POEMS综合征的可能性。

Objective To deepen the understanding of multi-system damage caused by POEMS (polyneuropathy,organomegaly,endocrinopathy,M protein elevation,and skin changes syndrome) in clinical practice by reporting a POEMS syndrome patient with diffuse proliferative glomerulonephritis.Methods Clinical data about one POEMS syndrome patient with diffuse proliferative glomerulonephritis admitted to Liyuan Hospital in October 2,2018 were retrospectively analyzed and related literatures were reviewed.Results The case was a 70 year-old man with edema and hypertension for 5 months.Simultaneously,symptoms including multiple lymphadenopathy,osteosclerosis,serosal effusion,double M proteinemia,and Bence-Jones proteinuria were also found in this patient.Histopathological findings of kidney and lymph nodes indicated diffuse proliferative glomerulonephritis and transparent vascular Castleman disease,respectively.Initially,the patient was misdiagnosed as Castleman’s disease combined with diffuse proliferative glomerulonephritis,and then he was treated with thalidomide.However,the symptoms did not improve significantly.Subsequently,pericardial effusion and weakness of both lower limbs appeared.Then we reviewed related literatures,and realized that it might be POEMS syndrome which we neglected before.Further tests revealed peripheral neuropathy and increased vascular endothelial growth factor (VEGF,1 423.08 pg/ml↑),confirming the diagnosis of POEMS syndrome.After given RCOP (rituximab +cyclophosphamide + vinorelbine + prednisone) chemotherapy for 6 months,the symptoms were significantly improved,and VEGF level decreased.Conclusion Glomerulonephritis caused by POEMS syndrome is rare and prone to occur in patients with Castleman disease.Therefore,POEMS syndrome should not be overlooked on the basis that kidney damage and Castleman disease coexist.