Stojanovic Katia Stankovic, Georgin-Lavialle Sophie, Grateau Gilles
Service de médecine interne, centre de référence des amyloses d'origine inflammatoire et de la fièvre méditerranéenne familiale, hôpital Tenon, 4, rue de la Chine, 75020 Paris, France; Département hospitalo-universitaire inflammation immunopathologie biothérapie (DHU I2B), faculté de médecine, université Pierre-et-Marie-Curie, 4, rue de la Chine, 75020 Paris, France.
Service de médecine interne, centre de référence des amyloses d'origine inflammatoire et de la fièvre méditerranéenne familiale, hôpital Tenon, 4, rue de la Chine, 75020 Paris, France; Département hospitalo-universitaire inflammation immunopathologie biothérapie (DHU I2B), faculté de médecine, université Pierre-et-Marie-Curie, 4, rue de la Chine, 75020 Paris, France.
Nephrol Ther. 2017 Jun;13(4):258-264. doi: 10.1016/j.nephro.2017.03.001. Epub 2017 Apr 24.
AA amyloidosis remains one of the three main types of systemic amyloidosis with AL and ATTR. Its incidence has been however decreasing recently in Western countries. Chronic inflammatory diseases are currently the first cause of AA amyloidosis, including rheumatoid arthritis, spondyloarthritis and autoinflammatory diseases. Castleman's disease is a specific cause of AA amyloidosis that can be cured by surgery. A chronic inflammatory response is required to develop amyloidosis. Other genetic and environmental factors are also involved. The first clinical manifestation is a chronic glomerular nephropathy, which can be detected by urine examination and serum creatinine measure. Immunohistochemistry is mandatory to confirm the clinical diagnosis of AA amyloidosis and to avoid misdiagnosis. Long-term prognosis remains poor on chronic dialysis in case of clinical gut involvement. Current treatment is based on the control of the inflammatory response. Specific treatment aimed at inhibiting amyloid formation targeting serum amyloid P component and heparan sulphate are currently evaluated.
AA淀粉样变性仍然是与AL和ATTR淀粉样变性并列的三种主要系统性淀粉样变性类型之一。然而，其发病率最近在西方国家呈下降趋势。慢性炎症性疾病目前是AA淀粉样变性的首要病因，包括类风湿性关节炎、脊柱关节炎和自身炎症性疾病。卡斯特曼病是AA淀粉样变性的一种特殊病因，可通过手术治愈。淀粉样变性的发生需要慢性炎症反应。其他遗传和环境因素也有影响。最初的临床表现是慢性肾小球肾病，可通过尿液检查和血清肌酐测量来检测。免疫组织化学对于确诊AA淀粉样变性的临床诊断以及避免误诊是必不可少的。如果临床出现肠道受累，长期透析的预后仍然很差。目前的治疗基于对炎症反应的控制。目前正在评估针对血清淀粉样蛋白P成分和硫酸乙酰肝素抑制淀粉样蛋白形成的特异性治疗方法。
