Prevention and Treatment of Interstitial Lung Disease in Systemic Sclerosis
试验速览
- 阶段
- 不适用
- 发起方
- 入组人数
- 1,372
- 试验地点
- 11
- 主要终点
- Proportion of patients with 10% decline in FVC
研究概览
简要总结
Systemic sclerosis (SSc) is an orphan, multiorgan disease affecting the connective tissue of the skin and all internal organs. Interstitial lung disease is a frequent morbidity and mortality-driving manifestation in systemic sclerosis.
This observational trial (OT) is part of the collaborative project "DeSScipher", one out of five OTs to decipher the optimal management of systemic sclerosis. Aim of this observational try is to identify:
- The state of clinical practice in Europe for prevention and treatment of interstitial lung disease and its impact on lung function and disease progression
- The potential predictors and confounders for response to therapy
详细描述
Patients are routinely evaluated every 3 months over a 12-months period by medical history, physical examination, pulmonary function tests, VAS lung score and SF-36, SHAQ. Also, their medication and possible medication changes will be recorded.
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Prospective
入排标准
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Diagnosis fo SSc according to the ACR/EULAR criteria for adult or the PRES/ACR/EULAR criteria for juvenile SSc patients
- •SSc patients with proven ILD (by X-ray or CT scan)
- •Treatment with standard dosages according to current practice with (i) cyclophosphamide, (ii) azathioprine, (iii) mycophenolate mofetil, (iv) methotrexate, or (v) no therapy
- •Exclusion Criterion:
- •Patients with previous exposure to silica or asbestos
排除标准
- 未提供
结局指标
主要结局
Proportion of patients with 10% decline in FVC
时间窗: 1 year
The proportion of patients with ILD progression as defined by a 10% decline in FVC within 1 year of therapy
次要结局
- The need for oxygen support(1 year)
- The time to a 15% decline in DLCO or a drop <55% of predicted lung function(1 year)
- The mortality due to lung fibrosis(1 year)
研究者
Gabriela Riemekasten
Prof. Dr. med. Gabriela Riemekasten
Charite University, Berlin, Germany