Hsu Jung-Hui, Fang Yu-Wei, Yang An-Hung, Tsai Ming-Hsein
Division of Gastroenterology, Department of Internal Medicine Division of Nephrology, Department of Internal Medicine, Shin Kong Wu Ho-Su Memorial Hospital Fu-Jen Catholic University School of Medicine Ultrastructural and Molecular Pathology, Department of Pathology, Taipei Veterans General Hospital, Taipei Taiwan (ROC).
Medicine (Baltimore). 2018 Sep;97(37):e12416. doi: 10.1097/MD.0000000000012416.
Membranoproliferative glomerulonephritis (MPGN) can be induced by autoimmune diseases, chronic infection, chronic hepatitis, and paraproteins (including cryoglobulinemia). In addition, the mixed cryoglobulinemic MPGN is reported to be highly correlated with hepatitis C virus (HCV) infection.
We reported a rare case of a 61-year-old woman without a history of viral hepatitis infection; she presented with bilateral leg edema and proteinuria. Renal pathology revealed MPGN with multiple positive immunofluorescent staining. The consequent serum survey revealed positive cryoglobulin and monoclonal gammopathy of kappa type of immunoglobulin M. However, bone marrow study showed no obvious plasma cell proliferation, indicating that multiple myeloma was less likely.
This patient's cryoglobulinemic MPGN could be related to monoclonal gammopathy of undetermined significance.
Oral immunosuppressant.
After steroid treatment, her renal function normalized and proteinuria kept in low level.
We demonstrated a rare cause of cryoglobulinemic MPGN without HCV infection, which led to a favorable prognosis after receiving steroid therapy. Moreover, the diagnosis of monoclonal gammopathy should be considered when facing such case and aggressive steroid therapy might be beneficial.
膜增生性肾小球肾炎(MPGN)可由自身免疫性疾病、慢性感染、慢性肝炎和副蛋白(包括冷球蛋白血症)诱发。此外,据报道,混合性冷球蛋白血症性MPGN与丙型肝炎病毒(HCV)感染高度相关。
我们报告了一例罕见病例,一名61岁女性,无病毒性肝炎感染史;她出现双侧腿部水肿和蛋白尿。肾脏病理显示为MPGN,免疫荧光染色呈多处阳性。随后的血清检查显示冷球蛋白阳性和κ型免疫球蛋白M单克隆丙种球蛋白病。然而,骨髓检查未发现明显的浆细胞增殖,表明多发性骨髓瘤的可能性较小。
该患者的冷球蛋白血症性MPGN可能与意义未明的单克隆丙种球蛋白病有关。
口服免疫抑制剂。
经过类固醇治疗后,她的肾功能恢复正常,蛋白尿维持在低水平。
我们证明了一例无HCV感染的冷球蛋白血症性MPGN的罕见病因,接受类固醇治疗后预后良好。此外,面对此类病例时应考虑单克隆丙种球蛋白病的诊断,积极的类固醇治疗可能有益。