Proposal of podocytic infolding glomerulopathy as a new disease entity: a review of 25 cases from nationwide research in Japan
Proposal of podocytic infolding glomerulopathy as a new disease entity: a review of 25 cases from nationwide research in Japan
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DOI：
10.1007/s10157-008-0104-z
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发表时间：
2008-12-01
影响因子：
2.3
通讯作者：
Makino, Hirofumi
中科院分区：
文献类型：
--
作者：
Joh, Kensuke;Taguchi, Takashi;Makino, Hirofumi
背景一种罕见而特殊的肾小球病在日本已开始被认识。日本肾脏病学会已经建立了一个研究工作组，并收集了来自日本各地的病例，试图了解完整的光谱，这肾小球pathology.Method的诊断标准，这是需要收集的情况下，提出了肾小球病变显示微球或微管结构或与足细胞折叠成肾小球基底膜（GBM）的电子显微镜。高碘酸乌洛托品银染色显示肾小球基底膜上有一个非嗜银孔，与膜性肾小球肾炎相似。患者年龄20-69岁（19名女性，6名男性）。17名患者还患有胶原蛋白疾病，如狼疮性肾炎和干燥综合征。所有患者均有蛋白尿。23例患者中有15例在12个月内蛋白尿得到缓解，但5例患者尽管接受了不同类型的治疗，蛋白尿仍高于1.0 g/天。包括微球在内的足细胞内折叠显示免疫球蛋白阳性或阴性染色。17例胶原病患者中有4例发现微球簇形成，8例无胶原病患者中有5例发现微球簇形成。17例胶原病患者中有6例GBM可见电子致密沉积物，而8例无胶原病患者GBM未见电子致密沉积物。结论部分患者可能为狼疮性肾炎、Ⅴ类或膜性肾小球肾炎的亚型。然而，我们提出了一种新的疾病实体，足细胞内折叠肾小球病，作为所有25例患者的共同基础，因为我们怀疑微球或微管结构或两者都可以来自足细胞内折叠。
Background A rare and peculiar glomerulopathy has begun to be recognized in Japan. The Japanese Society of Nephrology has established a research working group and has collected cases from all over Japan in an attempt to understand the complete spectrum of this glomerulopathy.Method The diagnostic criterion, which was needed to collect the cases, was proposed as a glomerulopathy showing microspheres or microtubular structures or both associated with podocytic infolding into the glomerular basement membrane (GBM) on electron microscopy. The lesion shows a non-argentaffin hole in the GBM with periodic acid methenamine silver staining and is similar to membranous glomerulonephritis.Results Twenty-five cases were collected from 17 institutions. Patients were 20-69 years old (19 women, 6 men). Seventeen patients also had collagen diseases such as lupus nephritis and Sjogren's syndrome. All patients had proteinuria. Proteinuria showed a remission in 15 of 23 patients within 12 months, but proteinuria remained higher than 1.0 g/day in five patients despite different types of therapy. Podocytic infolding including microspheres showed either positive or negative staining for immunoglobulins. Cluster formation of microspheres was found in 4 of 17 patients with collagen disease, and in five out eight patients without collagen disease. Electron-dense deposits in the GBM were also found in 6 of 17 patients with collagen disease but were not found in eight patients without collagen disease.Conclusion Some patients might have a subtype of lupus nephritis, class V, or membranous glomerulonephritis. However, we propose a new disease entity, podocytic infolding glomerulopathy, as a common basis of all 25 patients, because we suspect that microspheres or microtubular structures or both can be derived from podocytic infolding.