^(11)C-PiB PET/MRI在原发性系统性轻链型淀粉样变器官受累评估中的价值

Value of^(11)C-PiB PET/MRI in the evaluation of organ involvement in primary systemic light chain amyloidosis

目的分析^(11)C标记的匹兹堡化合物B(^(11)C-PiB)PET/MRI在原发性系统性轻链型淀粉样变(pAL)中评估器官受累的价值。方法回顾性分析2019年1月至2021年10月在华中科技大学同济医学院附属协和医院就诊的20例pAL患者及3名健康志愿者的临床资料,比较临床标准评估器官受累和PET/MRI评估的相关性,分析心脏相关生物学指标、疾病分期与心脏最大标准摄取值(SUVmax)之间的关系及24 h尿蛋白定量与肾脏SUVmax之间的关系。结果①纳入20例患者,初诊患者18例,非初诊患者2例。观察到^(11)C-PiB摄取阳性的脏器分别为:心脏15例(75%),肺部8例(40%),骨髓10例(50%),肌肉10例(50%),舌肌7例(35%),甲状腺6例(30%),唾液腺4例(20%),脾脏2例(10%),胃壁1例(5%)。②^(11)C-PiB在心脏及骨髓摄取的阳性率与临床评估标准具有很好的相关性。在肺组织、脾脏、腺体、肌肉和舌肌中,^(11)C-PiB PET/MRI评估的阳性率明显高于临床标准。但对于神经系统受累和脂肪组织的评估^(11)C-PiB PET/MRI具有局限性。③分析18例初诊患者心脏相关生物学指标与心脏SUVmax之间的关系。左心室射血分数(LVEF)<50%且室间隔厚度(ISV)≥1.2 cm的患者相较LVEF≥50%且ISV<1.2 cm的患者其心脏SUVmax更高(P<0.05)。心脏SUVmax值在Mayo2004分期、Mayo2012分期各期之间差异有统计学意义,分期越晚,SUVmax值越高(P<0.05)。心脏SUVmax与心肌肌钙蛋白酶Ⅰ、N末端前体脑钠肽水平呈明显正相关(P<0.01),肾脏SUVmax与24 h尿蛋白定量无明显相关性(P>0.05)。结论全身^(11)C-PiB PET/MRI作为一种淀粉样蛋白的可视化系统,若用于器官水平的定性评估,具有提高早期无创性诊断pAL水平的潜力;用于器官水平(尤其是心脏)的定量评估,有望更精准评估器官功能和预测疾病预后。

Objective To analyze the value of^(11)C-PiB PET/MRI for evaluating organ involvement in patients with primary light chain amyloidosis(pAL).Methods The clinical data of 20 patients with pAL and 3 healthy volunteers from January 2019 to October 2021 were retrospectively analyzed.The correlation between the organ involvement evaluated by clinical standards and PET/MRI was compared.The relationship between cardiac-related biological indicators,disease stage,and the maximum standardized uptake value(SUVmax)were analyzed.The relationship between 24-hour urinary protein quantification and kidney SUVmax was analyzed.Results①In 20 patients(18 newly diagnosed patients and 2 non-newly diagnosed patients),^(11)C-PiB positive uptake was observed in the heart(15 patients,75%),lung(8 patients,40%),bone marrow(10 patients,50%),muscle(10 patients,50%),tongue muscle(7 patients,35%),thyroid(6 patients,30%),salivary gland(4 patients,20%),spleen(2 patients,10%),and stomach wall(1 patient,5%).②Organ involvement on^(11)C-PiB PET/MRI showed good correlations with the clinical evaluation criteria for the heart and bone marrow.The positive rate of PET/MRI evaluation in the lung,spleen,gland,muscle,and tongue muscle was significantly higher than the clinical criteria.However,^(11)C-PiB PET/MRI has limitations in the evaluation of the nervous system and fat tissue.③To analyze the relationship between cardiac-related biological indexes and the SUVmax of the heart in 13 newly diagnosed patients.Patients with left ventricular ejection fraction(LVEF)<50%and interventricular septal thickness(ISV)≥1.2 cm showed a higher SUVmax than patients with LVEF≥50%and ISV<1.2 cm(P<0.05).There are significant differences in the SUVmax of the heart between the Mayo2004 stage and the Mayo2012 stage.The later the disease stage,the higher the SUVmax(P<0.05).The SUVmax of the heart was positively correlated with cardiac troponin I(cTnI)and N-terminal pro-brain natriuretic peptide(NT-proBNP)(P<0.01).There was no significant correlation between renal SUVmax and 24-hour urine protein(P>0.05).Conclusion Whole body^(11)C-PiB PET/MRI,as a visualization system of amyloid protein,is used to qualitatively evaluate organ involvement,which can improve the level of early noninvasive diagnosis.Whole body^(11)C-PiB PET/MRI can be used to perform quantitative evaluation of organ levels,especially the heart,which is expected to evaluate organ function and predict disease prognosis more accurately.