Abstract:
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. Results: ①In 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 non-invasive 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.
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。.