Effect of Bosentan in the Course of Scleroderma Renal Crisis
试验速览
- 阶段
- 2 期
- 状态
- 已完成
- 入组人数
- 16
- 试验地点
- 2
- 主要终点
- To explore the efficacy of bosentan (Tracleer) in patients with scleroderma renal crisis on renal function
研究概览
简要总结
Systemic sclerosis (SSc) is a connective tissue disease characterized by excessive collagen deposition, autoimmunity and by vascular hyper-reactivity and obliterative microvascular phenomena that involves multiple organs. Scleroderma Renal Crisis (SRC) occurs in 5% of patients and mainly with diffuse cutaneous SSc. The routine use of angiotensin-converting enzyme inhibitors (ACEI) has been reported to dramatically improve outcome, with a fall of the 12-month mortality from 76% to less than 15% in the United-States. Despite prognostic improvement, SRC remains a severe manifestation of SSc and functional outcome and survival remains poor. Bosentan is a specific, orally active, dual endothelin receptor antagonist that has recently been approved for the treatment of primary pulmonary arterial hypertension and for the prevention of ischemic digital ulcers. Bosentan could have therapeutic benefits on others vascular injuries and particularly in SRC.
详细描述
Systemic sclerosis (SSc) is a connective tissue disease characterized by excessive collagen deposition, autoimmunity and by vascular hyper-reactivity and obliterative microvascular phenomena that involves multiple organs. Scleroderma Renal Crisis (SRC) occurs in 5% of patients and mainly with diffuse cutaneous SSc. The routine use of angiotensin-converting enzyme inhibitors (ACEI) has been reported to dramatically improve outcome, with a fall of the 12-month mortality from 76% to less than 15% in the United-States. Despite prognostic improvement, SRC remains a severe manifestation of SSc and functional outcome and survival remains poor. Bosentan is a specific, orally active, dual endothelin receptor antagonist that has recently been approved for the treatment of primary pulmonary arterial hypertension and for the prevention of ischemic digital ulcers. Bosentan could have therapeutic benefits on others vascular injuries and particularly in SRC.
研究设计
- 研究类型
- Interventional
- 分配方式
- Na
- 干预模型
- Single Group
- 主要目的
- Treatment
- 盲法
- None
入排标准
- 年龄范围
- 18 Years 至 —（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Men or women ≥ 18 years
- •Patients had to fulfil ACR and/or LEROY et MEDSGER criteria for systemic sclerosis
- •Patients had to fulfil criteria for renal systemic sclerosis
- •Written informed consent obtained
排除标准
- •Scleroderma renal crisis occuring before the age of eighteen
- •Patients who are receiving bosentan within one month of inclusion for pulmonary arterial hypertension or digital ulcers prevention
- •Other treatment by selective or nonselective antagonist endothelin receptor
- •Left ventricle systolic dysfunction (EF < 40 %)
- •Patients with systolic blood pressure < 85mm Hg
- •Progressive cancer or considered cured for less than 5 years
- •Patients with a known hypersensitivity to bosentan or any of the excipients
- •Patients with HIV, HCV, HBV infection
- •Patients with Liver disease Child-Pugh B and C
- •Patients who are pregnant or breast-feeding
- •Women of child-bearing age who are sexually active without practising reliable methods of contraception
- •Patients who do not give informed consent
研究组 & 干预措施
Bosentan
Bosentan 62.5mg bid x 4 weeks; up-titrated to 125mg bid x 20 weeks
干预措施: Bosentan (Drug)
结局指标
主要结局
To explore the efficacy of bosentan (Tracleer) in patients with scleroderma renal crisis on renal function
时间窗: 6 and 12 months
次要结局
- To evaluate the safety and tolerability of bosentan in patients with scleroderma renal crisis over 6 months of treatment(6 months)
- 1 year overall survival(1 year)