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伴有大量线圈样病变的IV型狼疮性肾炎的快速完全缓解

Rapid and Complete Remission of Class IV Lupus Nephritis with Massive Wire Loop Lesions.

作者信息

Suzuki Tomo, Matsumura Ryutaro, Kitamura Hiroshi, Shibagaki Yugo

机构信息

Division of Nephrology and Hypertension, Department of Internal Medicine, St. Marianna University School of Medicine, Kawasaki, Japan.

Department of Rheumatology and Allergy, Chiba-East National Hospital, Chiba, Japan.

出版信息

Case Rep Nephrol Dial. 2018 Mar 22;8(1):56-61. doi: 10.1159/000487920. eCollection 2018 Jan-Apr.

Abstract

Class IV lupus nephritis (LN) often has a poorer prognosis than other classes. However, class IV LN has various phenotypes, including not only segmental and global types but also others. We present the case of a 29-year-old woman with class IV-G LN who had an early response to glucocorticoid monotherapy. In addition, multiple lung nodules such as miliary tuberculosis (TB) were detected on computed tomography. All cultures of sputum, gastric fluid, and bone marrow were negative. A kidney biopsy revealed diffuse endocapillary proliferative glomerulonephritis with marked subendothelial deposition. Electron microscopy revealed massive electron-dense deposits in the subendothelial area, mesangium area, and peritubular capillaries. The histological diagnosis was class IV-G (A) LN. We administered high-dose glucocorticoid monotherapy. After treatment, the LN and the lung lesions had complete, rapid remission for 1 month. The lung lesions were associated with an immune complex similar to wire loop lesions, not TB. Thus, it is important to consider class IV-G LN with massive wire loop lesions as a new subtype.

摘要

IV 型狼疮性肾炎(LN)的预后通常比其他类型更差。然而,IV 型 LN 有多种表型,不仅包括节段性和球性类型,还包括其他类型。我们报告了一例 29 岁患有 IV - G 型 LN 的女性患者,该患者对糖皮质激素单一疗法有早期反应。此外,计算机断层扫描发现了多个肺结节,如粟粒性肺结核(TB)。痰、胃液和骨髓的所有培养结果均为阴性。肾脏活检显示弥漫性毛细血管内增生性肾小球肾炎,伴有明显的内皮下沉积。电子显微镜检查显示在内皮下区域、系膜区域和肾小管周围毛细血管中有大量电子致密沉积物。组织学诊断为 IV - G(A)型 LN。我们给予了高剂量糖皮质激素单一疗法。治疗后,LN 和肺部病变在 1 个月内完全、迅速缓解。肺部病变与类似于线圈样病变的免疫复合物有关,而非 TB。因此,将伴有大量线圈样病变的 IV - G 型 LN 视为一种新的亚型很重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6b68/5903134/f12994e0b2c9/cnd-0008-0056-g01.jpg

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