Tan S Y, Irish A, Winearls C G, Brown E A, Gower P E, Clutterbuck E J, Madhoo S, Lavender J P, Pepys M B, Hawkins P N
Department of Medicine, Royal Postgraduate Medical School, Hammersmith Hospital, London, United Kingdom.
Kidney Int. 1996 Jul;50(1):282-9. doi: 10.1038/ki.1996.313.
We report the five year outcome of nine patients with dialysis-related amyloid (DRA) who underwent successful renal transplantation (RT) and six patients who remained on hemodialysis (HD). Amyloid bone cysts, a radiologic feature of DRA, and scintigraphy with 123I-labeled serum amyloid P component (SAP), a specific technique for evaluating amyloid deposits in vivo, were monitored and compared with clinical features. In all HD patients there was clinical, scintigraphic and/or radiologic evidence that DRA progressed. In contrast, eight of the RT patients experienced profound early relief of DRA symptoms following transplantation that persisted throughout follow-up, despite the reduction or withdrawal of corticosteroids. Amyloid bone cysts improved in four patients and SAP scans demonstrated regression of articular amyloid in eight out of nine cases. The modest radiographic improvement suggests that amyloid is mobilized more slowly in bone cysts than elsewhere or that cystic bone is remodeled poorly. This is the first objective evidence that DRA regresses following renal transplantation, and suggests that this may contribute to the long-term relief of DRA symptoms in transplant recipients who discontinue corticosteroids.
我们报告了9例接受成功肾移植（RT）的透析相关性淀粉样变（DRA）患者和6例继续接受血液透析（HD）患者的五年结局。监测了DRA的放射学特征——淀粉样骨囊肿，以及用123I标记血清淀粉样蛋白P成分（SAP）进行的闪烁扫描（一种在体内评估淀粉样蛋白沉积的特异性技术），并将其与临床特征进行比较。所有HD患者均有临床、闪烁扫描和/或放射学证据表明DRA病情进展。相比之下，8例RT患者在移植后早期DRA症状得到明显缓解，且在整个随访过程中持续存在，尽管减少或停用了皮质类固醇。4例患者的淀粉样骨囊肿有所改善，9例中有8例的SAP扫描显示关节淀粉样变消退。影像学上的适度改善表明，淀粉样蛋白在骨囊肿中的移动比在其他部位更慢，或者囊性骨的重塑较差。这是肾移植后DRA病情消退的首个客观证据，表明这可能有助于长期缓解停用皮质类固醇激素的移植受者的DRA症状。
