Arnaud Laurent, Malek Zoulikha, Archambaud Frédérique, Kas Aurélie, Toledano Dan, Drier Aurélie, Zeitoun Delphine, Cluzel Philippe, Grenier Philippe A, Chiras Jacques, Piette Jean-Charles, Amoura Zahir, Haroche Julien
Hôpital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, and Université Paris 6, Paris, France.
Arthritis Rheum. 2009 Oct;60(10):3128-38. doi: 10.1002/art.24848.
Erdheim-Chester disease (ECD) is a rare form of non-Langerhans' cell histiocytosis. The aim of this study was to assess the value of whole-body scanning with (18)F-fluorodeoxyglucose-positron emission tomography (FDG-PET) in a large cohort of ECD patients from a single center.
We retrospectively reviewed all PET scans performed on 31 patients with ECD who were referred to our department between 2005 and 2008. PET images were reviewed by 2 independent nuclear medicine specialist physicians and were compared with other imaging modalities performed within 15 days of each PET scan.
Thirty-one patients (10 women and 21 men; median age 59.5 years) underwent a total of 65 PET scans. Twenty-three patients (74%) were untreated at the time of the initial PET scan, whereas 30 of the 34 followup PET scans (88%) were performed in patients who were undergoing immunomodulatory therapy. Comparison of the initial and followup PET scans with other imaging modalities revealed that the sensitivity of PET scanning varied greatly among the different organs studied (range 4.3-100%), while the specificity remained high (range 69.2-100%). Followup PET scans were particularly helpful in assessing central nervous system (CNS) involvement, since the PET scan was able to detect an early therapeutic response of CNS lesions, even before magnetic resonance imaging showed a decrease in their size. PET scanning was also very helpful in evaluating the cardiovascular system, which is a major prognostic factor in ECD, by assessing the heart and the entire vascular tree during a single session.
The results of our large, single-center, retrospective study suggest that the findings of a FDG-PET scan may be interesting in the initial assessment of patients with ECD, but its greater contribution is in followup of these patients.
厄德里希-切斯特病（ECD）是一种罕见的非朗格汉斯细胞组织细胞增多症。本研究的目的是评估在一个单中心的大量ECD患者队列中，采用（18）F-氟脱氧葡萄糖正电子发射断层扫描（FDG-PET）进行全身扫描的价值。
我们回顾性分析了2005年至2008年间转诊至我科的31例ECD患者所进行的所有PET扫描。PET图像由2名独立的核医学专科医生进行评估，并与每次PET扫描后15天内进行的其他成像检查进行比较。
31例患者（10例女性和21例男性；中位年龄59.5岁）共接受了65次PET扫描。23例患者（74%）在首次PET扫描时未接受治疗，而34次随访PET扫描中的30次（88%）是在接受免疫调节治疗的患者中进行的。将首次和随访PET扫描与其他成像检查进行比较发现，PET扫描在不同研究器官中的敏感性差异很大（范围为4.3%-100%），而特异性仍然很高（范围为69.2%-100%）。随访PET扫描在评估中枢神经系统（CNS）受累方面特别有用，因为PET扫描能够检测到CNS病变的早期治疗反应，甚至在磁共振成像显示其大小减小之前。PET扫描在评估心血管系统方面也非常有帮助，心血管系统是ECD的一个主要预后因素，它可以在一次检查中评估心脏和整个血管树。
我们这项大型单中心回顾性研究的结果表明，FDG-PET扫描的结果在ECD患者的初始评估中可能有意义，但其更大的贡献在于对这些患者的随访。
