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[过敏性紫癜性肾炎合并原发性混合性冷球蛋白血症病例]

[Case of Henoch-Schönlein purpura nephritis complicated with essential mixed cryoglobulinemia].

作者信息

Kanashiki Eriko, Nakazawa Eiko, Akimoto Tetsu, Kato Maki, Saito Osamu, Ando Yasuhiro, Muto Shigeaki, Kusano Eiji

机构信息

Division of Nephrology, Department of Internal Medicine, Jichi Medical University, Tochigi, Japan.

出版信息

Nihon Jinzo Gakkai Shi. 2008;50(8):1024-9.

Abstract

We report a case of purpura nephritis complicated with essential mixed cryoglobulinemia. The patient was referred to our hospital because of a petechial rash on the lower extremities, microscopic hematuria, and progressive deterioration of renal function. The presumptive diagnosis of Henoch-Schönlein purpura (HSP) was made, and the patient was treated with prednisolone at the dose of 40 mg/day. However, there was a persistent purpuric skin rash. On the other hand, immunoelectrophoresis of the serum revealed the presence of IgA-lambda and polyclonal IgG in the cryoprecipitate. Granular staining for polyclonal rather than monoclonal IgA and C3 segmentally along the capillary walls demonstrated by immunofluorescence analysis of renal biopsy led to the diagnosis of purpura nephritis as the major mechanism of renal damage. After three sessions of cryofiltration, the patient's serum cryoglobulins decreased and the active rash finally settled, along with improvement of renal function. These observations suggest that the presence of cryoglobulinemia modulated the clinical course of HSP in our case. Therefore, the possibility of the latent presence of cryoglobulinemia in cases with HSP having an active rash refractory to steroid treatment should not be overlooked.

摘要

我们报告一例紫癜性肾炎合并原发性混合性冷球蛋白血症。患者因下肢瘀点疹、镜下血尿及肾功能进行性恶化转诊至我院。初步诊断为过敏性紫癜(HSP),患者接受了40mg/天的泼尼松龙治疗。然而,皮肤紫癜持续存在。另一方面,血清免疫电泳显示冷沉淀物中存在IgA-λ和多克隆IgG。肾活检免疫荧光分析显示沿毛细血管壁呈节段性多克隆而非单克隆IgA和C3颗粒状染色,提示紫癜性肾炎是肾脏损伤的主要机制。经过三次冷滤过后,患者血清冷球蛋白减少,活动性皮疹最终消退,肾功能也有所改善。这些观察结果表明,冷球蛋白血症的存在改变了我们病例中HSP的临床病程。因此,对于皮疹活跃且对类固醇治疗无效的HSP病例,不应忽视潜在冷球蛋白血症的可能性。

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