Tominaga Ayako, Wada Keiji, Kato Yoshiharu, Okazaki Ken
Department of Orthopedic Surgery, Tokyo Women's Medical University, Tokyo, 162-8666, Japan.
Department of Orthopedic Surgery, Kita Shinagawa 3rd Hospital, Tokyo, 140-0001, Japan.
JBMR Plus. 2024 Nov 22;9(1):ziae154. doi: 10.1093/jbmrpl/ziae154. eCollection 2025 Jan.
Calciphylaxis, also known as calcific uremic arteriolopathy (CUA), is a rare disorder with many unknown treatment and diagnostic aspects. It is characterized by calcification and thrombosis of small blood vessels. This disease leads to progressive skin calcification, necrotizing ulcers, and infections and is associated with a high mortality rate. Although primarily affected sites tend to be on skin, those affecting bones are also significant. We report a case of CUA complicated with rapidly progressing multiple vertebral fractures and severe osteoporosis. The patient experienced a series of five vertebral fractures within 5 months after hospitalization, and blood tests revealed abnormally high levels of bone resorption marker bone-type tartrate-resistant acid phosphatase (TRACP-5b). Consequently, intravenous sodium thiosulfate and hyperbaric oxygen therapy were administered for the treatment of skin lesions caused by calciphylaxis, and brace therapy and denosumab treatment were initiated for vertebral fractures. This approach rapidly decreased TRACP-5b levels and arrested the chain of vertebral fractures. We concluded that to maintain the quality of life of patients with CUA, early treatment of primary skin lesions as well as comorbid conditions is essential.
钙过敏,也称为钙化性尿毒症性小动脉病(CUA),是一种罕见疾病,在治疗和诊断方面有许多未知之处。其特征是小血管钙化和血栓形成。这种疾病会导致皮肤逐渐钙化、坏死性溃疡和感染,并伴有高死亡率。虽然主要受累部位往往在皮肤,但影响骨骼的情况也很严重。我们报告一例CUA并发快速进展的多发性椎体骨折和严重骨质疏松症的病例。患者在住院后5个月内发生了一系列5次椎体骨折,血液检查显示骨吸收标志物骨型抗酒石酸酸性磷酸酶(TRACP-5b)水平异常升高。因此,给予静脉注射硫代硫酸钠和高压氧治疗以治疗钙过敏引起的皮肤病变,并开始对椎体骨折进行支具治疗和地诺单抗治疗。这种方法迅速降低了TRACP-5b水平,并阻止了椎体骨折的连锁反应。我们得出结论,为维持CUA患者的生活质量,早期治疗原发性皮肤病变以及合并症至关重要。