The first experience of denosumab therapy on patients with Ehlers-Danlos syndrome and osteoporosis: detailed observation of two patients.
The first experience of denosumab therapy on patients with Ehlers-Danlos syndrome and osteoporosis: detailed observation of two patients.
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狄诺塞麦治疗埃勒斯-当洛斯综合征合并骨质疏松症患者的初体验：两名患者的详细观察
DOI：
10.1080/24725625.2020.1799493
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发表时间：
2021
期刊：
影响因子：
--
通讯作者：
Takahashi J.
中科院分区：
文献类型：
--
作者：
Yasukawa S;Uehara M;Suzuki T;Nakano M;Kosho T;Nakamura Y;Takahashi J.
先前的研究表明，埃勒斯-当洛斯综合征 (EDS) 患者的骨矿物质密度 (BMD) 下降是由于未知机制所致，并描述了 EDS 骨质疏松症的治疗方法。然而，迄今为止，还没有确定的治疗方法。在本研究中，我们调查了两名 EDS 患者，以阐明狄诺塞麦治疗 EDS 骨质疏松症患者的有效性和安全性。我们回顾性纳入了两名接受狄诺塞麦治疗的 EDS 骨质疏松症患者。患者 1 是一名 59 岁男性，患有典型的 EDS 和骨质疏松症，接受了 48 个月的地诺塞麦治疗。治疗前腰椎骨密度和髋部总骨密度分别为1.335 g/cm2和0.762 g/cm2。 48 个月后，腰椎和总髋部 BMD 分别增加了 1.6% 和 11.4%。患者 2 是一名 42 岁男性，患有血管性 EDS 和骨质疏松症，接受了 18 个月的地诺塞麦治疗。治疗前腰椎骨密度和髋部总骨密度分别为0.763 g/cm2和0.583 g/cm2。 18 个月后，腰椎和总髋部 BMD 分别增加了 5.0% 和 1.8%。观察期间，两例病例均未出现骨折或其他并发症。这是 denosmab 治疗 EDS 合并骨质疏松症患者的首次经验。狄诺塞麦安全给药，没有骨折等严重不良反应，对 BMD 产生波动但可能积极的影响，可能成为这些患者的一种治疗选择。
Previous studies have shown decreased bone mineral density (BMD) due to an unknown mechanism in Ehlers-Danlos Syndrome (EDS) patients and described approaches to treatment for osteoporosis in EDS. To date, however, there is no established method of treatment. In this study, we investigated two patients with EDS to clarify the efficacy and safety of denosumab treatment in EDS patients with osteoporosis. We retrospectively enrolled two EDS patients with osteoporosis who underwent denosumab therapy. Patient 1 was a 59-year-old male with classical EDS and osteoporosis who received a 48-month course of denosumab therapy. His lumbar BMD and total hip BMD were 1.335 g/cm2and 0.762 g/cm2before treatment, respectively. Forty-eight months later, the lumbar and total hip BMD showed gains of 1.6% and 11.4%, respectively. Patient 2 was a 42-year-old male with vascular EDS and osteoporosis who received an 18-month course of denosumab therapy. His lumbar BMD and total hip BMD were 0.763 g/cm2and 0.583 g/cm2before treatment, respectively. Eighteen months later, the lumbar and total hip BMD showed gains of 5.0% and 1.8%, respectively. No fractures or other complications were recorded during the observational period in both cases. This is the first experience of denosmab therapy on patients with EDS and osteoporosis. Denosumab, administered safely with no serious adverse effects such as fractures, exerted a fluctuating but probably positive effect regarding BMD and could be a treatment option on these patients.