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[AA淀粉样变性的诊断与治疗]

[Diagnostics and therapy of AA amyloidosis].

作者信息

Blank N, Lorenz H M

机构信息

Medizinische Klinik V, Sektion Rheumatologie, Universitätsklinikum Heidelberg, Im Neuenheimer Feld 410, 69120, Heidelberg, Deutschland.

出版信息

Pathologe. 2009 May;30(3):219-25. doi: 10.1007/s00292-009-1140-5.

Abstract

AA amyloidosis can be the consequence of any chronic inflammatory disorder. It is most commonly associated with chronic inflammatory rheumatic, pulmonary or gastrointestinal diseases, familial Mediterranean fever or other rare periodic syndromes. AA amyloidosis often affects the kidneys, gastrointestinal tract and the heart. Effective therapy of the underlying disease can normalize the inflammatory reaction and can slow or inhibit the deterioration of organ function if the diagnosis is made at an early stage of the disease. In rheumatoid diseases and in some periodic syndromes the use of antibodies against TNFalpha or IL-1 beta might be helpful. Patients with familial Mediterranean fever should regularly take colchicine to prevent attacks and to reduce the risk for development or progression of AA amyloidosis. Eprodisate is currently being investigated for AA amyloidosis and renal involvement.

摘要

AA型淀粉样变性可能是任何慢性炎症性疾病的后果。它最常与慢性炎症性风湿性、肺部或胃肠道疾病、家族性地中海热或其他罕见的周期性综合征相关。AA型淀粉样变性常累及肾脏、胃肠道和心脏。如果在疾病早期作出诊断,对基础疾病进行有效的治疗可使炎症反应正常化,并可减缓或抑制器官功能的恶化。在类风湿性疾病和一些周期性综合征中,使用抗TNFα或IL-1β抗体可能会有帮助。家族性地中海热患者应定期服用秋水仙碱以预防发作,并降低AA型淀粉样变性发生或进展的风险。目前正在对依普硫酸钠治疗AA型淀粉样变性和肾脏受累情况进行研究。

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