Cuchard P, Cuchard R, Rotman S, Burnier M, Gauthier T
Hôpital Riviera, Site de la Providence, Vevey.
Rev Med Suisse. 2012 Feb 29;8(330):446-51.
Amyloidosis is defined as the extracellular deposition of proteins that have the capacity to form beta-pleated sheets and become insoluble. More than 17 types of amyloidosis have been described. Systemic light chain amyloid (AL) and AA amyloid (secondary to chronic inflammatory process) are by far the most frequent forms of amyloidosis. In these systemic forms, organs involved are the kidneys, the heart and the gastrointestinal tract in AL amyloidosis. The diagnostic can be established only by tissue biopsy. Treatment of primary amyloidosis (AL) aims at suppressing the responsible clone whereas treatment of secondary amyloidosis relies on controlling the underlying inflammatory process. Prognosis is globally poor and depends on the extend of organs involvement particularly cardiac and renal. The prognosis is even worse in patients requiring dialysis.
淀粉样变性病的定义为具有形成β折叠片层并变得不溶能力的蛋白质在细胞外沉积。已描述了超过17种类型的淀粉样变性病。系统性轻链淀粉样蛋白（AL）和AA淀粉样蛋白（继发于慢性炎症过程）是迄今为止最常见的淀粉样变性病形式。在这些系统性形式中，AL淀粉样变性病累及的器官是肾脏、心脏和胃肠道。仅通过组织活检才能确诊。原发性淀粉样变性病（AL）的治疗旨在抑制相关克隆，而继发性淀粉样变性病的治疗则依赖于控制潜在的炎症过程。总体预后较差，取决于器官受累的程度，尤其是心脏和肾脏。需要透析的患者预后更差。
