Blank N, Hegenbart U, Schönland S
Amyloidose-Zentrum, Universitätsklinikum Heidelberg, Im Neuenheimer Feld 410, 69120, Heidelberg, Deutschland.
Z Rheumatol. 2016 Mar;75(2):141-50. doi: 10.1007/s00393-015-0024-8.
Systemic amyloidoses are rare protein deposition disorders, which are often diagnosed in an advanced stage of the disease due to non-specific symptoms. Any chronic inflammatory disease can lead to an AA-type amyloidosis.
This paper summarizes the current state of the art of diagnosis and treatment of AA amyloidosis and presents data from the past 10 years of our amyloidosis center.
Our data represents an analysis of our cohort of patients with amyloidosis and a selective research in the PubMed database for AA amyloidosis.
The underlying diseases comprise autoinflammatory syndromes, polyarthritis, and chronic inflammatory bowel and lung diseases. Renal organ involvement is the most prevalent in AA amyloidosis. It can be detected early through the evaluation of proteinuria. The treatment depends on the individual underlying disease. Patients without an associated inflammatory disease are considered to have idiopathic AA amyloidosis and empiric treatment is mandatory.
Survival of this fatal disease has recently improved due to the new diagnostic tools and treatment options; however, early diagnosis plays a crucial role in the prevention of end-stage renal failure. New therapeutic strategies aim to remove existing amyloid deposits.
系统性淀粉样变性是罕见的蛋白质沉积疾病，由于症状不具特异性，常在疾病晚期才得以诊断。任何慢性炎症性疾病均可导致AA型淀粉样变性。
本文总结了AA型淀粉样变性诊断与治疗的当前技术水平，并展示了我们淀粉样变性中心过去10年的数据。
我们的数据包括对淀粉样变性患者队列的分析以及在PubMed数据库中对AA型淀粉样变性的选择性研究。
潜在疾病包括自身炎症综合征、多关节炎以及慢性炎症性肠病和肺病。肾脏器官受累在AA型淀粉样变性中最为常见。通过评估蛋白尿可早期检测到。治疗取决于个体潜在疾病。无相关炎症性疾病的患者被视为患有特发性AA型淀粉样变性，必须进行经验性治疗。
由于新的诊断工具和治疗选择，这种致命疾病的生存率最近有所提高；然而，早期诊断在预防终末期肾衰竭中起着关键作用。新的治疗策略旨在清除现有的淀粉样沉积物。
