Connective Tissue Diseases and Lung Manifestations Prospective Trial With Focus on Systemic Sclerosis (Colipris)
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 发起方
- 入组人数
- 120
- 试验地点
- 1
- 主要终点
- Increase in the modified Rodnan Skin Score
研究概览
简要总结
Despite a number of prospective studies already initiated in the past years, the current epidemiology and course of interstitial lung disease (ILD) and pulmonary hypertension (PH) in patients with connective tissue disease (CTD) is still not well defined, particularly regarding its prevalence, incidence and the management of a broad spectrum of disease presentations.
Major challenges include the identification of patients with progressive disease, the appropriate time point of therapeutic intervention and the underlying driver of disease (inflammatory or pro-fibrotic stimulus or both?).
To address these issues in Western Austria, a progressive registry of patients with CTD exploring routine clinical and pathophysiological characteristics of ILD and PH will be conducted. This multidisciplinary, prospective and observational registry aims to collect comprehensive clinical data on incidence, prevalence and course of disease regarding all PH and ILD presentations in a real-world setting.
详细描述
Specifically, this registry will collect demographic data, disease-related clinical data, routine laboratory values (including antibody-profile and iron status), diagnostic procedures, significant comorbidities, therapeutic managements (e.g. thoracic ultrasound, HRCT), and disease outcomes over 10 years.
In addition, blood biobank samples for translational research will be collected in a subgroup of patients with systemic sclerosis over serial time points to study the systemic inflammatory and profibrotic phenotype of patients. In summary, this registry will monitor the disease course of pulmonary manifestations of patients with CTD and may be hypothesis-generating and provide new insights in underlying inflammatory/pro-fibrotic patterns.
研究设计
- 研究类型
- Observational
- 观察模型
- Other
- 时间视角
- Prospective
入排标准
- 年龄范围
- 18 Years 至 80 Years（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •The inclusion criteria are adult patients (≥18 years) diagnosed with CTD with signed informed consent.
排除标准
- •Exclusion criteria are patients with a serious co-morbidity interfering with the course of interstitial lung disease or pulmonary hypertension.
结局指标
主要结局
Increase in the modified Rodnan Skin Score
时间窗: 1 year
Change in the modified Rodnan Skin Score from baseline of \>5 Points assessed by a trained professional
Decline in Diffusion capacity for carbon monoxide
时间窗: 1 year
Absolute decline from baseline in diffusion capacity for carbon monoxide in percent measured. Lung function tests are performed by a trained professional.
Decline in forced vital capacity
时间窗: 1 year
Absolute decline from baseline in forced vital capacity percent predicted. Lung function tests are performed by a trained professional.
次要结局
未报告次要终点