A Prospective Observational Registry to Describe the Disease Course and Outcomes of Idiopathic Pulmonary Fibrosis Patients in a Real-world Clinical Setting. PROOF-Registry New and Extended Belgium -Luxembourg
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 发起方
- 入组人数
- 575
- 试验地点
- 9
- 主要终点
- Number of participants alive
研究概览
简要总结
A Prospective Observational Registry to describe the disease course and outcomes of Idiopathic Pulmonary Fibrosis patients in a real-world clinical setting.
详细描述
Idiopathic pulmonary fibrosis (IPF) is a chronic disease of unknown etiology, characterized by scar tissue (fibrosis) within the lungs. IPF is one of the most common forms of interstitial lung disease and is associated with substantial morbidity and mortality (average survival of approximately three years from the time of diagnosis)).
The epidemiology and natural history of IPF is still not completely understood. Incidence and prevalence of IPF are difficult to determine because uniform diagnostic criteria have only recently been defined. Recent data among subjects aged 50 years or older suggests an age- and sex-adjusted incidence rate ranging from 8.8 cases to 17.4 cases per 100,000 person-years in the USA. In the same population, age- and sex- adjusted prevalence ranged from 27.9 cases to 63 cases per 100,000 persons.
Familial form of IPF accounts for 0.5 to 2% of all cases of IPF. Several environmental or occupational exposures seem to be prevalent in the medical histories of patients diagnosed with the disease.
The onset of IPF symptoms is slow, but symptoms become progressively worse over time. Breathlessness upon exertion and chronic dry cough are the major symptoms. Gastro-esophageal acid reflux is present in almost 90% of patients with IPF but often occurs without symptoms.
Auscultation of the lungs reveals early inspiratory crackles, predominantly located in the lower posterior lung zones upon physical exam. Clubbing is found in approximately 50% of patients with IPF.
研究设计
- 研究类型
- Observational
- 观察模型
- Case Only
- 时间视角
- Prospective
入排标准
- 年龄范围
- 19 Years 至 —（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Patients >18 years of age
- •IPF diagnosis "definite" or "probable" (2011 ATS/ERS guidelines definitions) confirmed by a multidisciplinary team (= minimum of pulmonologist, radiologist, pathologist, all with expertise in IPF).
- •Patients who agreed to participate in the registry and have completed and signed the Informed Consent Form
排除标准
- •Patients incapable of giving informed consent
- •Patients participating in a clinical trial at the time of inclusion in the registry and whose protocol does not allow participation in another trial
结局指标
主要结局
Number of participants alive
时间窗: overall duration of study (5 years)
Data on death will be retrieved from the medical file of the participant.
The first occurrence of a decrease of ≥ 15% in percent predicted diffusing capacity of the lung for carbon monoxide (DLCO) since baseline
时间窗: overall duration of study (5 years)
DLCO data will be retrieved from the medical file of the participant.
The evolution of percent predicted forced vital capacity (FVC)
时间窗: overall duration of study (5 years)
FVC data will be retrieved from the medical file of the participant.
The number of acute exacerbations of IPF
时间窗: overall duration of study (5 years)
Acute exacerbations of IPF data will be retrieved from the medical file of the participant.
The first occurrence of a decrease of ≥ 10% in percent predicted forced vital capacity (FVC) since baseline.
时间窗: overall duration of study (5 years)
FVC data will be retrieved from the medical file of the participant.
The evolution of percent predicted diffusing capacity of the lung for carbon monoxide (DLCO)
时间窗: overall duration of study (5 years)
DLCO data will be retrieved from the medical file of the participant.
次要结局
- The number of patients per IPF treatment drug(overall duration of study (5 years))
- The mean number of dose changes per IPF treatment drug(overall duration of study (5 years))
- The number of participants with lung transplantation(overall duration of study (5 years))
- The mean initial dose per IPF treatment drug(overall duration of study (5 years))
- The mean duration of drug interruption in days per IPF treatment drug(overall duration of study (5 years))
- The impact of IPF and IPF treatment on quality of life measured with with the K-BILD questionnaire(overall duration of study (5 years))
- The number of participants who have discontinued the IPF treatment per IPF treatment drug(overall duration of study (5 years))
- The mean duration of dose reduction in days per IPF treatment drug(overall duration of study (5 years))
- The (S)ADRs per IPF treatment reported which are possibly or probably related to the IPF treatment drug.(overall duration of study (5 years))
- The impact of IPF and IPF treatment on the severity of cough measured with the Cough-Visual Analogue Scale.(overall duration of study (5 years))
- The mean duration of treatment in days per IPF treatment drug(overall duration of study (5 years))
- The utilisation of health care resources by participants(overall duration of study (5 years))
- The impact of IPF and IPF treatment on quality of life measured with the SGRQ.(overall duration of study (5 years))
- The impact of IPF and IPF treatment on quality of life measured with the EQ-5D-5L questionnaire.(overall duration of study (5 years))