EVALUATION OF SYSTEMIC AMYLOIDOSIS BY SCINTIGRAPHY WITH I-123 LABELED SERUM AMYLOID-P COMPONENT
EVALUATION OF SYSTEMIC AMYLOIDOSIS BY SCINTIGRAPHY WITH I-123 LABELED SERUM AMYLOID-P COMPONENT
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DOI：
10.1056/nejm199008233230803
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发表时间：
1990-08-23
影响因子：
158.5
通讯作者：
PEPYS, MB
中科院分区：
文献类型：
--
作者：
HAWKINS, PN;LAVENDER, JP;PEPYS, MB
背景：在系统性淀粉样变性中，疾病的分布和进展一直很难监测，因为它们只能通过活检来证实。血清淀粉样蛋白P组分(SAP)是一种正常的循环血浆蛋白，由于其与淀粉样蛋白纤维的特异性结合而沉积在淀粉样蛋白纤维上。我们调查了标记SAP是否可以用来定位淀粉样蛋白沉积。方法：对50例经活检证实的系统性淀粉样变性患者(AL(原发型)25例，AA(继发性)型25例)、26例对照组和10例健康者静脉注射碘-123标记的纯化人SAP。24小时后获得全身图像和区域视图，并以盲读方式阅读。结果：在淀粉样变性患者中，123I-SAP快速定位于淀粉样蛋白沉积。获得的闪烁图像具有特征性，似乎能识别淀粉样蛋白沉积的范围。获得的闪烁图像具有特征性，似乎识别了所有50名患者的淀粉样蛋白沉积的程度。对照组和健康人均未摄取~(123)I-SAP。在所有AA淀粉样变性患者中，脾都受到了影响，而扫描显示只有AL型患者的心脏、皮肤、腕骨区域和骨髓有摄取。在6名活检阴性或不成功的患者中发现了阳性图像；在所有6名患者中，随后在活检或尸检中发现了淀粉样蛋白。在连续研究的11例患者中，有9例观察到进行性淀粉样蛋白沉积。结论：注射~(123)I-SAP后的核素扫描可用于诊断、定位和监测系统性淀粉样变性的程度。
Background: In systemic amyloidosis the distribution and progression of disease have been difficult to monitor, because they can be demonstrated only by biopsy. Serum amyloid P component (SAP) is a normal circulating plasma protein that is deposited on amyloid fibrils because of its specific binding affinity for them. We investigated whether labeled SAP could be used to locate amyloid deposits. Methods: Purified human SAP labeled with iodine-123 was given intravenously in 50 patients with biopsy-proved systemic amyloidosis-25 with the AL (primary) type and 25 with the AA (secondary) type- and to 26 control patients with disease and 10 healthy subjects. Whole-body images and regional views were obtained after 24 hours and read in a blinded fashion. Results: In the patients with amyloidosis the 123I-SAP was localized rapidly and specifically in amyloid deposits. The scintigraphic images obtained were characteristic and appeared to identify the extent of amyloid deposits. The scintographic images obtained were characteristic and appeared to identify the extent of amyloid deposition in all 50 patients. There was no uptake of the 123I-SAP by the control patients and the healthy subjects. In all patients with AA amyloidosis the spleen was affected, whereas the scans showed uptake in the heart, skin, carpal region, and bone marrow only in patients with the AL type. Positive images were seen in six patients in whom biopsies have been negative or unsuccessful; in all six, amyloid was subsequently found on biopsy or at autopsy. Progressive amyloid deposition was observed in 9 of 11 patients studied serially. Conclusions: Scintigraphy after the injection of 123I-SAP can be used for diagnosing, locating, and monitoring the extent of systemic amyloidosis.