未经授权:卡努拉蒂-恩格尔曼病(CED)的治疗涉及使用糖皮质激素,镇痛药,和双膦酸盐;使用氯沙坦的经验是有限的。我们的目的是描述被诊断为CED的患者的情况,该患者在使用类固醇和双膦酸盐时症状仍然难治,并成功使用氯沙坦治疗。
未经证实:一名27岁的妇女出现骨痛累及四肢和大关节1年。临床检查显示骨压痛和近端肌病,1型胶原的C末端肽升高(1617pg/mL;正常范围,137-573pg/mL)和1型前胶原水平的N端前肽(163ng/mL;正常范围,5.1-58.3ng/mL)。钙(9.4毫克/分升;正常范围,8.3-10.4mg/dL),磷酸盐(3.4毫克/分升;正常范围,2.5-4.5mg/dL),和甲状旁腺激素(62pg/mL;正常范围,8-80pg/mL)的水平在正常范围内。射线照片显示增生累及下肢和上肢长骨的骨干区域,并做出了CED的临时诊断。她接受了强的松龙治疗,每天30毫克,她报告说有一些改善。由于泼尼松龙,她出现了外源性库欣综合征,它的剂量逐渐减少。随后,她的骨痛加重了。此后,她开始口服阿仑膦酸钠。由于持续的疼痛,添加氯沙坦,之后骨痛明显减轻,1型胶原的C端肽(375pg/mL)和1型前胶原的N端前肽(50ng/mL)水平降低.
未经批准:偶尔,CED提出了治疗挑战,当它的症状对常规剂量的类固醇和双膦酸盐仍然难以治疗时,可能需要其他选择。上述患者开始服用氯沙坦,其通过下调转化生长因子β1起作用,导致疼痛减轻。
UNASSIGNED:氯沙坦下调转化生长因子β1,如果症状仍然难以治疗,氯沙坦可作为CED患者的类固醇保留选择。
UNASSIGNED: The treatment of Camurati-Engelmann disease (CED) involves the use of glucocorticoids, analgesics, and bisphosphonates; experience with the use of losartan is limited. Our objective was to describe the case of a patient diagnosed with CED whose symptoms remained refractory while on steroids and bisphosphonates and who was successfully treated with losartan.
UNASSIGNED: A 27-year-old woman presented with bone pain involving her extremities and large joints for 1 year. Clinical examination revealed bone tenderness and proximal myopathy with elevated C-terminal peptide of type 1 collagen (1617 pg/mL; normal range, 137-573 pg/mL) and N-terminal propeptide of type 1 procollagen levels (163 ng/mL; normal range, 5.1-58.3 ng/mL). Calcium (9.4 mg/dL; normal range, 8.3-10.4 mg/dL), phosphate (3.4 mg/dL; normal range, 2.5-4.5 mg/dL), and parathyroid hormone (62 pg/mL; normal range, 8-80 pg/mL) levels were within the normal range. Radiographs showed hyperostosis involving the diaphyseal region of long bones of the lower and upper limbs, and a provisional diagnosis of CED was made. She was treated with prednisolone, 30 mg daily, with which she reported some improvement. As exogenous Cushing syndrome had developed in her because of prednisolone, its dose was tapered. Subsequently, her bone pain worsened. Thereafter, she was initiated on oral alendronate. Due to persistent pain, losartan was added, after which she had marked decrease in bone pain with a reduction in the C-terminal peptide of type 1 collagen (375 pg/mL) and N-terminal propeptide of type 1 procollagen (50 ng/mL) levels.
UNASSIGNED: Occasionally, CED presents therapeutic challenges, and when its symptoms remain refractory to conventional doses of steroids and bisphosphonates, other options may be needed. The abovementioned patient was initiated on losartan, which acts by downregulation of transforming growth factor β1, leading to the reduction in pain.
UNASSIGNED: Losartan downregulates transforming growth factor β1 and may be offered as a steroid-sparing option in individuals diagnosed with CED if symptoms remain refractory to conventional treatment.