Department of Nephrology, Hitachi General Hospital, Japan.
Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan.
Intern Med. 2022 Jul 15;61(14):2191-2196. doi: 10.2169/internalmedicine.8714-21. Epub 2022 Mar 12.
Polyneuropathy, Organomegaly, Endocrinopathy, M-protein and Skin changes (POEMS) syndrome manifests as elevated levels of vascular endothelial growth factor (VEGF) and monoclonal gammopathy. We treated a case of POEMS syndrome showing monoclonality in both IgA-λ and IgG-κ. Serial renal biopsies before treatment and after normalization of the VEGF levels suggested that glomerular microangiopathy had developed due to VEGF, while biclonal gammopathy was not eliminated. The renal pathology, proteinuria, and renal function all clearly improved. Although severe polyneuropathy limited activities of daily living and enforced a bedridden state, the patient dramatically regained his motor function, achieving crutch walking after induction of remission. This case is highly notable due to the presence of biclonality and repeated biopsies.
POEMS 综合征表现为血管内皮生长因子（VEGF）水平升高和单克隆丙种球蛋白病，其特征为多发性神经病、器官肿大、内分泌病、M 蛋白和皮肤改变。我们治疗了一例 IgA-λ 和 IgG-κ 均表现为单克隆性的 POEMS 综合征。治疗前和 VEGF 水平正常化后的连续肾活检提示，VEGF 导致肾小球微血管病，而双克隆丙种球蛋白病未消除。肾脏病理、蛋白尿和肾功能均明显改善。尽管严重的多发性神经病限制了日常生活活动并导致卧床不起，但患者在诱导缓解后显著恢复了运动功能，实现了用拐杖行走。由于存在双克隆性和重复活检，该病例非常值得注意。
