Evolution of heterotopic bone in fibrodysplasia ossificans progressiva: An [18F]NaF PET/CT study
Evolution of heterotopic bone in fibrodysplasia ossificans progressiva: An [18F]NaF PET/CT study
复制标题
DOI：
10.1016/j.bone.2019.03.009
复制
发表时间：
2019-07-01
期刊：
影响因子：
4.1
通讯作者：
Eekhoff, E. Marelise W.
中科院分区：
文献类型：
--
作者：
Botman, Esmee;Raijmakers, Pieter G. H. M.;Eekhoff, E. Marelise W.
进行性纤维发育不良骨化症(FOP)是一种罕见的常染色体显性遗传性疾病，其特征是肌肉、韧带和肌腱的异位骨化(HO)。在HO形成之前，通常会出现突发事件，导致关节不能活动。由于疾病的进展没有突发的迹象，推测HO的慢性进展并存，但缺乏确凿的证据。最近，有研究表明，[F-18]NaF PET/CT能够在突发疾病发作期间识别早期骨化疾病活动。因此，本研究的目的是评估[F-18]NaF PET/CT是否也能识别FOP中可能存在的慢性进行性HO。扫描是在0.5到2年的时间里获得的。通过手工分割CT图像获得HO体积和标准摄取值(SUV)。获得了太阳/峰值，定义为包含最热体素像素的1毫升球体的平均SUV值。5名患者中有2名在[F-18]NAF PET/CT扫描时经历了1次活跃的临床发作。此外，在5名患者中，有4名在没有临床发作的情况下，通过CT体积评估，连续扫描显示HO的放射学进展(3-8厘米(3))。在已鉴定的HO结构中，有6/47(12.8%)出现这种体积增加，在所有病例中，伴随着[F-18]NaF摄取的增加，SUV峰值从8.4到17.9。[F-18]NAF PET/CT能够识别这些无症状的进行性HO病变，从而显示FOP慢性活动性的存在。因此，未来的药物不仅应该针对新的HO形成，而且应该针对这种慢性HO进展。
Fibrodysplasia ossificans progressiva (FOP) is a rare, autosomal dominant disorder characterized by heterotopic ossification (HO) in muscles, ligaments and tendons. Flare-ups often precede the formation of HO, resulting in immobilization of joints. Due to progression of the disease without signs of a flare-up, co-existence of a chronic progression of HO has been postulated, but conclusive evidence is lacking. Recently, it has been shown that [F-18] NaF PET/CT is able to identify early ossifying disease activity during flare-ups. Therefore, the purpose of the present study was to assess whether [F-18]NaF PET/CT might also be able to identify the possible presence of chronic progressive HO in FOP.A total of thirteen [F-18]NaF PET/CT scans from five FOP patients were analysed. Scans were acquired over a period of 0.5 to 2 years. Volumes of HO and standardized uptake values (SUV) were obtained based on manual segmentation of CT images. SUN/peak values, defined as the average SUV value of a 1 mL sphere containing the hottest voxel pixels, were obtained.Two out of five patients experienced 1 active clinical flare-ups at the time of the [F-18]NaF PET/CT scan. In addition, in four out of five patients, serial scans showed radiological progression of HO (3 to 8 cm(3)), as assessed by CT volume, in the absence of a clinical flare-up. This volumetric increase was present in 6/47 (12.8%) of identified HO structures and, in all cases, was accompanied by increased [F-18]NaF uptake, with SUVpeak ranging from 8.4 to 17.9.In conclusion, HO may progress without signs of a flare-up. [F-18]NaF PET/CT is able to identify these asymptomatic, but progressive HO lesions, thereby demonstrating the presence of chronic activity in FOP. Consequently, future drugs should not only target new HO formation, but also this chronic HO progression.