Department of Internal Medicine Section Endocrinology, VU University Medical Center, Amsterdam, The Netherlands.
Department of Internal Medicine Section Endocrinology, VU University Medical Center, Amsterdam, The Netherlands.
Bone. 2018 Apr;109:143-146. doi: 10.1016/j.bone.2017.08.012. Epub 2017 Aug 18.
Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disease with a progressive course characterized by episodically local flare-ups, which often but not always leads to heterotopic bone formation (HO). Recently, we showed that [18F]NaF PET/CT may be the first tool to monitor progression of a posttraumatic flare-up leading to new HO, which was demonstrated in a patient with FOP who underwent a maxillofacial surgery. This paper evaluates [18F]NaF PET/CT as a marker of FOP disease activity, comparing its use with other imaging modalities known in literature. In addition, the follow-up of a spontaneous flare-up in a 19-year old patient is presented showing high muscle [18F]NaF uptake in one defined part within the flare-up area after three weeks. During follow-up [18F]NaF PET /CT scan revealed newly formed heterotopic bone but only in this previously active [18F]NaF region. In conclusion, increased muscle [18F]NaF uptake may predict future HO development in FOP patients. At present [18F]NaF PET/CT appears to be a sensitive imaging modality to serve as a noninvasive marker for bone formation and to monitor disease activity during flare-ups in FOP.
进行性骨化性纤维发育不良(FOP)是一种罕见的遗传性疾病,具有进行性病程,其特征为间歇性局部发作,通常但并非总是导致异位骨形成(HO)。最近,我们表明[18F]NaF PET/CT 可能是监测导致新 HO 的创伤后发作进展的第一个工具,在接受颌面外科手术的 FOP 患者中得到了证实。本文评估了[18F]NaF PET/CT 作为 FOP 疾病活动的标志物,将其与文献中已知的其他成像方式进行了比较。此外,还介绍了一名 19 岁患者自发性发作的随访情况,显示在发作后 3 周,发作区域内一个明确部位的肌肉[18F]NaF 摄取量增加。在随访期间,[18F]NaF PET/CT 扫描显示新形成的异位骨,但仅在先前活跃的[18F]NaF 区域。总之,肌肉[18F]NaF 摄取增加可能预示 FOP 患者未来发生 HO。目前,[18F]NaF PET/CT 似乎是一种敏感的成像方式,可作为骨形成的非侵入性标志物,并在 FOP 发作期间监测疾病活动。