Chacko Liza, Boldrini Michele, Martone Raffaele, Law Steven, Martinez-Naharrro Ana, Hutt David F, Kotecha Tushar, Patel Rishi K, Razvi Yousuf, Rezk Tamer, Cohen Oliver C, Brown James T, Srikantharajah Mukunthan, Ganesananthan Sharmananthan, Lane Thirusha, Lachmann Helen J, Wechalekar Ashutosh D, Sachchithanantham Sajitha, Mahmood Shameem, Whelan Carol J, Knight Daniel S, Moon James C, Kellman Peter, Gillmore Julian D, Hawkins Philip N, Fontana Marianna
Division of Medicine, National Amyloidosis Centre (L.C., M.B., R.M., S.L., A.M.-N., D.F.H., T.K., R.K.P., Y.R., T.R., O.C.C., J.B., M.S., S.G., T.L., H.L., A.W., S.S., S.M., C.W., D.S.K., J.G., P.N.H., M.F.), University College London, London, United Kingdom.
Department of Internal Medicine, Amyloidosis Research and Treatment Center, IRCCS Policlinico San Matteo Foundation, Pavia, Italy (M.B.).
Circ Cardiovasc Imaging. 2021 Apr;14(4):e012506. doi: 10.1161/CIRCIMAGING.121.012506. Epub 2021 Apr 20.
Systemic amyloidosis is characterized by amyloid deposition that can involve virtually any organ. Splenic and hepatic amyloidosis occurs in certain types, in some patients but not others, and may influence prognosis and treatment. SAP (serum amyloid P component) scintigraphy is uniquely able to identify and quantify amyloid in the liver and spleen, thus informing clinical management, but it is only available in 2 centers globally. The aims of this study were to examine the potential for extracellular volume (ECV) mapping performed during routine cardiac magnetic resonance to: (1) detect amyloid in the liver and spleen and (2) estimate amyloid load in these sites using SAP scintigraphy as the reference standard.
Five hundred thirty-three patients referred to the National Amyloidosis Centre, London, between 2015 and 2017 with suspected systemic amyloidosis who underwent SAP scintigraphy and cardiac magnetic resonance with T1 mapping were studied.
The diagnostic performance of ECV to detect splenic and hepatic amyloidosis was high for both organs (liver: area under the curve, -0.917 [95% CI, 0.880-0.954]; liver ECV cutoff, 0.395; sensitivity, 90.7%; specificity, 77.7%; <0.001; spleen: area under the curve, -0.944 [95% CI, 0.925-0.964]; spleen ECV cutoff, 0.385; sensitivity, 93.6%; specificity, 87.5%; <0.001). There was good correlation between liver and spleen ECV and amyloid load assessed by SAP scintigraphy (r=0.504, <0.001; r=0.693, <0.001, respectively). There was high interobserver agreement for both the liver and spleen (ECV liver intraclass correlation coefficient, 0.991 [95% CI, 0.984-0.995]; <0.001; ECV spleen intraclass correlation coefficient, 0.995 [95% CI, 0.991-0.997]; <0.001) with little bias across a wide range of ECV values.
Our study demonstrates that ECV measurements obtained during routine cardiac magnetic resonance scans in patients with suspected amyloidosis can identify and measure the magnitude of amyloid infiltration in the liver and spleen, providing important clues to amyloid type and offering a noninvasive measure of visceral amyloid burden that can help guide and track treatment.
系统性淀粉样变性的特征是淀粉样蛋白沉积，几乎可累及任何器官。脾和肝淀粉样变性在某些类型的患者中出现，而在其他患者中不出现，并且可能影响预后和治疗。血清淀粉样蛋白P成分（SAP）闪烁扫描能够独特地识别和量化肝脏和脾脏中的淀粉样蛋白，从而为临床管理提供信息，但全球仅有两个中心可开展此项检查。本研究的目的是探讨在常规心脏磁共振成像过程中进行细胞外容积（ECV）测绘的潜力，以：（1）检测肝脏和脾脏中的淀粉样蛋白；（2）以SAP闪烁扫描作为参考标准，估计这些部位的淀粉样蛋白负荷。
对2015年至2017年间转诊至伦敦国家淀粉样变性中心的533例疑似系统性淀粉样变性患者进行了研究，这些患者均接受了SAP闪烁扫描和T1测绘的心脏磁共振成像检查。
ECV检测脾和肝淀粉样变性的诊断性能在两个器官中均较高（肝脏：曲线下面积，-0.917[95%CI，0.880-0.954]；肝脏ECV临界值，0.395；敏感性，90.7%；特异性，77.7%；<0.001；脾脏：曲线下面积，-0.944[95%CI，0.925-0.964]；脾脏ECV临界值，0.385；敏感性，93.6%；特异性，87.5%；<0.001）。肝脏和脾脏的ECV与通过SAP闪烁扫描评估的淀粉样蛋白负荷之间存在良好的相关性（r分别为0.504，<0.001；r为0.693，<0.001）。肝脏和脾脏的观察者间一致性均较高（肝脏ECV组内相关系数，0.991[95%CI，0.984-0.995]；<0.001；脾脏ECV组内相关系数，0.995[95%CI，0.991-0.997]；<0.001），在广泛的ECV值范围内偏差很小。
我们的研究表明，在疑似淀粉样变性患者的常规心脏磁共振扫描过程中获得的ECV测量值可以识别和测量肝脏和脾脏中淀粉样蛋白浸润的程度，为淀粉样蛋白类型提供重要线索，并提供一种无创的内脏淀粉样蛋白负荷测量方法，有助于指导和跟踪治疗。
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