Diagnostic performance of 123I-labeled serum amyloid P component scintigraphy in patients with amyloidosis

Diagnostic performance of 123I-labeled serum amyloid P component scintigraphy in patients with amyloidosis
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DOI:
10.1016/j.amjmed.2005.08.043
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发表时间:
2006-04-01
影响因子:
5.9
通讯作者:
Jager, PL
Jager, PL
中科院分区:
医学2区
文献类型:
--
作者:
Hazenberg, BPC;van Rijswijk, MH;Jager, PL

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目的:评价I-123标记血清淀粉样蛋白P组分(SAP)对系统性和局限性淀粉样变性患者诊断的准确性和提供的补充信息。对象和方法:将I-123标记的人SAP静脉注射给20例对照组和189例经组织学证实的淀粉样变性患者,其中AA型60例,AL型80例,遗传性ATTR型27例,局限性淀粉样变性22例。在注射示踪剂24小时后获得SAP闪烁图,并分析异常摄取模式。结果:SAP核素显像诊断系统性AA、AL和ATTR淀粉样变性的敏感性分别为90%、90%和48%,特异性为93%。淀粉样蛋白在AA中的分布不如在AL型中那么明显。所有患者均未观察到心肌摄取。脾淀粉样变在AA和AL型中非常常见(80%),但临床上很少发现(14%)。肝脏和肾脏的示踪剂摄取异常分别与肝功能紊乱和蛋白尿有关。骨髓摄取是AL特有的(21%),ALkappa比AL lambda更常见。结论:SAP核素显像对大多数AA和AL型患者的淀粉样蛋白有诊断作用,但遗传性ATTR型患者较少,这与淀粉样蛋白在这些疾病中的不同分布和负担有关。它通常比临床上发现的更广泛的器官受累,并且某些淀粉样蛋白的分布是特殊纤维类型的特征。(C)2006 Elsevier Inc.保留所有权利。
Purpose: To assess the diagnostic accuracy and additional information provided by I-123-labeled serum amyloid P component ( SAP) scintigraphy in patients with systemic and localized amyloidosis.Subjects and Methods: I-123-labeled human SAP was injected intravenously into 20 controls and 189 consecutive patients with histologically proven amyloidosis: of AA type in 60 cases, AL type in 80, hereditary ATTR type in 27, and localized amyloidosis in 22 cases. SAP scintigrams were obtained 24 hours after tracer injection and were analyzed for abnormal patterns of uptake. Sensitivity and specificity were determined, and scintigraphic findings were compared with clinical data.Results: Diagnostic sensitivity of SAP scintigraphy for systemic AA, AL, and ATTR amyloidosis was 90%, 90%, and 48% respectively, and specificity was 93%. The distribution of amyloid was less diverse in AA than in AL type. Myocardial uptake was not visualized in any patient. Splenic amyloid was very frequent (80%) in AA and AL type but rarely detected clinically (14%). Abnormal tracer uptake in the liver and kidneys correlated with disturbed liver function and proteinuria, respectively. Bone marrow uptake was specific for AL (21%) and was more frequent in AL kappa than AL lambda. Localized amyloid deposits were not imaged.Conclusion: SAP scintigraphy is diagnostic of amyloid in most patients with AA and AL type but fewer with hereditary ATTR type, relating to differing distributions and burdens of amyloid in these disorders. It usually reveals more widespread organ involvement than is identified clinically, and certain distributions of amyloid are characteristic of particular fibril types. (C) 2006 Elsevier Inc. All rights reserved.