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[淀粉样变性]

[Amyloidoses].

作者信息

Ladner-Merz S, Müller-Ladner U

机构信息

Akademie für Kognitives Training, Stuttgart, Deutschland.

出版信息

Z Rheumatol. 2008 Dec;67(8):677-82; quiz 683. doi: 10.1007/s00393-008-0385-3.

Abstract

Amyloidoses make up a group of diseases caused by misfolded proteins. These misfolded proteins are insoluble and are deposited in various tissues and organs, ultimately resulting in severe organ dysfunction. The majority of patients with amlyoidoses suffer from chronic inflammatory, infectious or malignant diseases. Moreover, unexplained nephropathy, cardiomyopathy, neuropathy, enteropathy, arthropathy or macroglossia with or without periorbital bleeding should include an amyloidosis in the differential diagnosis. The latter is facilitated by histological examination of abdominal adipose tissue, the rectum or affected organs. Therapy focuses predominantly on reduction of activity of the underlying disease and specific organ protection. More recent therapeutic strategies include interleukin-1 inhibition, as well as inhibitors of protein misfolding.

摘要

淀粉样变性是一组由错误折叠的蛋白质引起的疾病。这些错误折叠的蛋白质不溶,并沉积在各种组织和器官中,最终导致严重的器官功能障碍。大多数淀粉样变性患者患有慢性炎症、感染或恶性疾病。此外,原因不明的肾病、心肌病、神经病变、肠病、关节病或巨舌症,无论有无眶周出血,在鉴别诊断中都应考虑淀粉样变性。通过对腹部脂肪组织、直肠或受影响器官进行组织学检查有助于诊断。治疗主要侧重于降低基础疾病的活性和特定器官的保护。最近的治疗策略包括抑制白细胞介素-1以及抑制蛋白质错误折叠。

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