Characterization of IgA Deposition in the Kidney of Patients with IgA Nephropathy and Minimal Change Disease
Characterization of IgA Deposition in the Kidney of Patients with IgA Nephropathy and Minimal Change Disease
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DOI：
10.3390/jcm9082619
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发表时间：
2020-08-01
影响因子：
3.9
通讯作者：
Lee, Sang-Ho
中科院分区：
文献类型：
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作者：
Cho, Won-Hee;Park, Seon-Hwa;Lee, Sang-Ho
大约5%的伊加肾病（IgAN）患者表现出轻度系膜病变，伴有急性发作的肾病综合征和弥漫性足突消失，代表微小病变病（MCD）。目前尚不清楚这些罕见的IgAN伴MCD（IgAN-MCD）病例是IgAN的变异型还是MCD患者中伊加的偶然沉积。在韩国18家医院的回顾性多中心队列研究中，我们分析了46例IgAN-MCD患者。排除毛细血管内增生、节段性硬化和新月体患者，比较IgAN-MCD与单纯MCD的临床特征和预后。此外，我们进行了半乳糖缺陷型IgA 1（KM 55）染色，以表征IgAN-MCD。在入组数据库的21，697例肾小球肾炎患者中，46例患者（0.21%）诊断为IgAN-MCD，1610例患者（7.4%）诊断为单纯MCD。46例IgAN-MCD患者占原发性IgAN患者（n= 7584）的0.6%。IgAN-MCD患者与单纯MCD患者的预后无差异。免疫荧光双标法显示伊加和KM 55在IgAN-MCD（n= 4）和原发性IgAN（n= 5）中均呈双阳性。然而，在4例狼疮性肾炎，系膜伊加沉积，但半乳糖缺陷型IgA 1（Gd-IgA 1）没有。这些发现表明IgAN-MCD是一种双重肾小球病，其中MCD叠加在可能惰性的IgAN上。我们通过KM 55染色证实IgAN-MCD是真正的IgAN，从而能够更好地表征疾病。此外，IgAN-MCD在根据通常的MCD治疗方式治疗时显示出良好的预后。
Approximately 5% of patients with IgA nephropathy (IgAN) exhibit mild mesangial lesions with acute onset nephrotic syndrome and diffuse foot process effacement representative of minimal change disease (MCD). It is not clear whether these unusual cases of IgAN with MCD (IgAN-MCD) are variant types of IgAN or coincidental deposition of IgA in patients with MCD. In a retrospective multicenter cohort study of 18 hospitals in Korea, we analyzed 46 patients with IgAN-MCD. Patients with endocapillary proliferation, segmental sclerosis, and crescent were excluded, and the clinical features and prognosis of IgAN-MCD were compared with those of pure MCD. In addition, we performed galactose-deficient IgA1 (KM55) staining to characterize IgAN-MCD. Among the 21,697 patients with glomerulonephritis enrolled in the database, 46 patients (0.21%) were diagnosed with IgAN-MCD, and 1610 patients (7.4%) with pure MCD. The 46 patients with IgAN-MCD accounted for 0.6% of primary IgAN patients (n= 7584). There was no difference in prognosis between patients with IgAN-MCD and those with only MCD. IgA and KM55 showed double positivity in all patients with IgAN-MCD (n= 4) or primary IgAN (n= 5) under double immunofluorescent staining. However, in four patients with lupus nephritis, mesangial IgA was deposited, but galactose-deficient-IgA1 (Gd-IgA1) was not. These findings suggest that IgAN-MCD is a dual glomerulopathy in which MCD was superimposed on possibly indolent IgAN. We confirmed by KM55 staining that IgAN-MCD is true IgAN, enabling better characterizations of the disease. Furthermore, IgAN-MCD shows a good prognosis when treated according to the usual MCD treatment modality.