Touchard Guy, Bridoux Frank, Goujon Jean-Michel
Service de néphrologie, service d'anatomie pathologique et unité de pathologie ultrastructurale, hôpital Jean-Bernard, centre hospitalier universitaire, Poitiers, France.
Nephrol Ther. 2005 Dec;1(6):355-64. doi: 10.1016/j.nephro.2005.10.001. Epub 2005 Nov 17.
Glomerulonephritis with organized immunoglobulin deposits are heterogeneous and may be encountered in a variety of renal disorders. It is particularly important to determine the exact types of immunoglobulin deposited and their monotypy/monoclonality, using specific anti-light chain conjugates and in some instances, anti-heavy chain subclass conjugates. The histologic pattern and identification of monotypic Ig deposits in renal tissue are in some cases sufficient for the diagnosis of AL amyloidosis, type I cryoglobulinic glomerulonephritis or "Randall-type" monoclonal immunoglobulin deposition disease (MIDD). Electron microscopy is particularly valuable for the precise localization of Ig deposits and for determing the pattern of deposition: organized, homogeneous (non-organized or granular) or mixed. New entities in the broad spectrum of Ig deposition diseases are emerging from electron microscopy studies of renal diseases. We propose here a comprehensive classification for diseases featuring the organized deposition in tissues of Ig.
伴有免疫球蛋白沉积的肾小球肾炎具有异质性,可在多种肾脏疾病中出现。使用特异性抗轻链结合物,在某些情况下还使用抗重链亚类结合物,来确定沉积的免疫球蛋白的确切类型及其单型性/单克隆性尤为重要。在某些病例中,肾脏组织中组织学模式及单型性Ig沉积物的鉴定足以诊断AL淀粉样变性、I型冷球蛋白性肾小球肾炎或“兰德尔型”单克隆免疫球蛋白沉积病(MIDD)。电子显微镜对于Ig沉积物的精确定位及确定沉积模式(有组织的、均匀的(无组织的或颗粒状的)或混合的)特别有价值。肾脏疾病的电子显微镜研究不断揭示出Ig沉积疾病广泛谱中的新病种。在此,我们针对以Ig在组织中有组织沉积为特征的疾病提出一种全面的分类方法。