Effectiveness of cryofiltration and mizoribine combination with oral steroid therapy in a patient with membranoproliferative glomerulonephritis due to essential cryoglobulinemia.
Effectiveness of cryofiltration and mizoribine combination with oral steroid therapy in a patient with membranoproliferative glomerulonephritis due to essential cryoglobulinemia.
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冷冻滤过和咪唑立宾联合口服类固醇治疗对原发性冷球蛋白血症导致的膜增生性肾小球肾炎患者的有效性。
DOI：
10.1007/s13730-019-00394-4
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发表时间：
2019
期刊：
影响因子：
1
通讯作者：
Fukami K
中科院分区：
文献类型：
--
作者：
Kurokawa Y;Koike K;Kaida Y;Ito S;Chiba H;Urae K;Moriyama T;Nakamura N;Imai T;Shibata R;Hazama T;Wakasugi D;Okuda S;Fukami K
一位65岁男性肾病综合征患者，因全身水肿加重及四肢紫癜入院。他有肾功能受损，血清补体水平低，类风湿因子水平升高。他的cryocellulin（单克隆IgM-κ和多克隆混合型IgG）呈阳性，肾活检结果显示膜增生性肾小球肾炎（MPGN）的组织特征。由于次要原因尚不清楚，他被诊断为原发性混合性冷球蛋白血症引起的MPGN。住院第20天，患者开始接受50 mg/天泼尼松龙（PSL）治疗。住院第43天，处方口服咪唑立宾（MZR），剂量为150 mg/天。在住院第49天，由于疾病对类固醇耐药，因此进行了冷冻过滤。治疗迅速降低尿蛋白水平。血清白蛋白和血清补体水平升高，治疗开始后约3个月达到完全缓解。PSL和MZR剂量分别逐渐减少至2 mg/天和100 mg/天，三年内没有任何cryocyanulinemia症状的复发或肾病综合征的复发。在这里，我们报告了一例原发性混合性冷球蛋白血症，我们可以通过在口服皮质类固醇和咪唑立宾免疫抑制剂治疗的基础上增加冷冻滤过来达到疾病的完全缓解，并可以维持很长一段时间。
A 65-year-old male patient with nephrotic syndrome was admitted to our hospital due to worsening systemic edema and purpura on the limbs. He had an impaired renal function, low serum complement level, and elevated rheumatoid factor level. He was positive for cryoglobulin (monoclonal IgM-κ and polyclonal mixed-type IgG), and the results of his kidney biopsy showed a tissue profile of membranoproliferative glomerulonephritis (MPGN). Due to the fact that the secondary cause was unclear, he was diagnosed with MPGN due to essential mixed cryoglobulinemia. On hospital day 20, he was initiated on 50 mg/day prednisolone (PSL). On hospital day 43, oral mizoribine (MZR) at a dose of 150 mg/day was prescribed. On hospital day 49, cryofiltration was performed because the disease was steroid resistant. The treatment promptly decreased urine protein levels. Serum albumin and serum complement levels increased, and complete remission was achieved approximately three months after the initiation of treatment. The PSL and MZR doses were gradually reduced to 2 mg/day and 100 mg/day, respectively, without any reemergence of the symptoms of cryoglobulinemia or relapse of the nephrotic syndrome for three years. Here, we report this case with essential mixed cryoglobulinemia in whom we could achieve complete remission of the disease by adding cryofiltration to the oral corticosteroid and immunosuppressant therapy with mizoribine and could maintain for a long time.