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系统性淀粉样变性在强直性脊柱炎患者中表现为慢性腹泻。

Systemic amyloidosis presenting as chronic diarrhea in a patient with ankylosing spondylitis.

机构信息

Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX, USA.

出版信息

J Clin Rheumatol. 2010 Jan;16(1):22-5. doi: 10.1097/RHU.0b013e3181c78c1a.

DOI:10.1097/RHU.0b013e3181c78c1a
PMID:20051751
Abstract

Secondary (AA) amyloidosis is a disease that is caused by systemic deposition of amyloid fibrils. Circulating serum amyloid A protein is an acute phase reactant and levels are therefore high during inflammatory states. Chronic elevation of serum amyloid A levels results in accumulation and deposition in various organs, leading to organ dysfunction. Unless the inciting inflammatory state can be controlled, the subsequent development of amyloidosis diminishes patient survival. We report a case of systemic amyloidosis that presented primarily as chronic diarrhea in a patient with ankylosing spondylitis. Unfortunately for the patient, the diagnosis of amyloidosis was delayed for years despite encounters with multiple physicians. Early medical intervention to control chronic inflammation is imperative and could prevent morbidity and mortality related to the development of secondary amyloidosis. Consideration of amyloidosis as a diagnosis in patients who have chronic uncontrolled inflammatory conditions is also important in preventing poor outcomes related to this disease.

摘要

继发性(AA)淀粉样变是一种由淀粉样纤维沉积引起的全身性疾病。循环中的血清淀粉样蛋白 A 是一种急性时相反应物,因此在炎症状态下水平较高。血清淀粉样蛋白 A 水平的慢性升高导致其在各种器官中蓄积和沉积,从而导致器官功能障碍。除非能控制激发炎症的状态,否则随后发生的淀粉样变会降低患者的生存率。我们报告了一例以强直性脊柱炎患者慢性腹泻为主要表现的系统性淀粉样变。不幸的是,尽管患者遇到了多位医生,但淀粉样变的诊断仍被延误了数年。早期进行医学干预以控制慢性炎症至关重要,可预防与继发性淀粉样变发展相关的发病率和死亡率。对于患有慢性未得到控制的炎症性疾病的患者,考虑淀粉样变作为诊断也很重要,可预防与这种疾病相关的不良结局。

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Clin Endosc. 2013 Nov;46(6):651-5. doi: 10.5946/ce.2013.46.6.651. Epub 2013 Nov 19.
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Variegate porphyria complicated by systemic AA amyloidosis: a case report.
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