Amyloidosis is a group of disorders characterized by a misfolded protein that deposits in organs and compromise their function. Clinician should have a high index of suspicion because in most cases, the clinical picture is non-specific. Typing of amyloid is of utmost importance and should be an integral part of accurately diagnosing a patient.

AL amyloidosis is the most common systemic amyloidosis in the western world in which the misfolded proteins are immunoglobulin light chains secreted by clonal plasma cells. New data about prognostication of AL amyloidosis patients are accumulating. The treatment goal is to eradicate the amyloidogenic plasma cell clone, by using high dose melphalan and/or novel agents (proteasome inhibitors, immunomodulatory drugs, monoclonal antibodies against CD38). Early diagnosis is important for effectively treating the patient as late diagnosis hampers chances for organ recovery.

ATTR amyloidosis is less recognized but is increasingly seen due to better recognition and improved diagnostic tools. New data about treatment options (patisiran, inotersen and tafamidis) have recently been published and are discussed.

淀粉样变性病是一组以蛋白质错误折叠为特征的疾病，蛋白质沉积在器官中并损害其功能。临床医生应该高度怀疑，因为在大多数情况下，临床表现是非特异性的。淀粉样蛋白的打字是最重要的，并且应该是准确诊断患者不可或缺的一部分。

AL淀粉样变性是西方世界中最常见的系统性淀粉样变性，其中错误折叠的蛋白质是克隆浆细胞分泌的免疫球蛋白轻链。有关AL淀粉样变性患者预后的新数据正在积累。治疗目标是通过使用高剂量马法兰和/或新型药物（蛋白酶体抑制剂，免疫调节药物，针对CD38的单克隆抗体）消除产生淀粉样蛋白的浆细胞克隆。早期诊断对于有效治疗患者很重要，因为晚期诊断会妨碍器官恢复的机会。

ATTR淀粉样变性病的认识较少，但由于更好的认识和改进的诊断工具而越来越多地见到。有关治疗选择（patisiran，inotossen和tafamidis）的新数据最近已经发布并进行了讨论。