Jadoul M, Garbar C, van Ypersele de Strihou C
Department of Nephrology, Cliniques Universitaires St. Luc, Université Catholique de Louvain, Brussels, Belgium.
Semin Dial. 2001 Mar-Apr;14(2):86-9. doi: 10.1046/j.1525-139x.2001.00037.x.
Histology remains the gold standard to diagnose beta(2)-microglobulin amyloidosis (A beta(2)M). Two diagnostic criteria are required: positive Congo red staining with typical birefringence under polarized light and immunostaining of amyloid deposits with a labeled anti-beta(2)M antibody. A beta(2)M is preferentially located in the joints. Small deposits are also found in various organs, mainly the heart and gastrointestinal tract. Pathologic studies have demonstrated a high prevalence of articular A beta(2)M early in the course of hemodialysis and peritoneal dialysis, antedating clinical manifestations by several years. The stages of beta(2)M amyloid formation have been delineated: beta(2)M amyloid deposits first on the surface of the cartilage, in the absence of macrophages (stage 1), and subsequently involves capsules and synovia (stage 2), with eventual recruitment of macrophages around large beta(2)M amyloid deposits (stage 3). Clinical manifestations are likely associated with the inflammation observed in stage 3. The factors triggering the fibrillar precipitation of beta(2)M remain unknown. Macrophages do not play a role: their presence is the consequence rather than the cause of beta(2)M amyloid deposits. Several substances coprecipitated with beta(2)M amyloid have been incriminated: highly sulfated glycosaminoglycans such as chondroitin or keratan sulfate, antiproteases such as alpha(2)-macroglobulin, and apolipoprotein E. As yet, no definitive conclusion has been reached.
组织学仍是诊断β2-微球蛋白淀粉样变性（Aβ2M）的金标准。需要两个诊断标准：刚果红染色在偏振光下呈典型双折射阳性，以及用标记的抗β2M抗体对淀粉样沉积物进行免疫染色。Aβ2M优先沉积于关节。在各种器官中也发现有小的沉积物，主要是心脏和胃肠道。病理研究表明，在血液透析和腹膜透析过程早期，关节Aβ2M的患病率很高，比临床表现提前数年。β2M淀粉样蛋白形成的阶段已被明确：β2M淀粉样蛋白首先沉积在软骨表面，此时没有巨噬细胞（1期），随后累及关节囊和滑膜（2期），最终在大的β2M淀粉样蛋白沉积物周围募集巨噬细胞（3期）。临床表现可能与3期观察到的炎症有关。触发β2M纤维状沉淀的因素仍然未知。巨噬细胞不起作用：它们的存在是β2M淀粉样蛋白沉积的结果而非原因。有几种物质与β2M淀粉样蛋白共沉淀，受到怀疑：高度硫酸化的糖胺聚糖，如硫酸软骨素或硫酸角质素；抗蛋白酶，如α2-巨球蛋白；以及载脂蛋白E。目前尚未得出明确结论。
