Retrospective Multicenter Case-control Study Comparing Chronic Beryllium Disease to Pulmonary Sarcoidosis
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 入组人数
- 200
- 试验地点
- 1
- 主要终点
- The phenotypic profile at inclusion will be based on clinical data
研究概览
简要总结
Inhalation of beryllium can induce specific sensitization and diffuse pulmonary granulomatosis called chronic beryllium disease (CBD). The clinical, radiographic, and anatomopathological features of CBD are very similar to those of sarcoidosis, another granulomatosis, making its diagnosis difficult. In addition, the progression of CBD is poorly understood. The investigators hypothesis is that there are specific clinical, biological, anatomopathological, and radiological presentation specificities of CBD, as well as a worse prognosis compared to pulmonary sarcoidosis.
详细描述
Inhalation of beryllium can induce specific sensitization and diffuse pulmonary granulomatosis called chronic beryllium disease (CBD). The clinical, radiographic, and anatomopathological features of CBD are very similar to those of sarcoidosis, another granulomatosis, making its diagnosis difficult. In addition, the progression of CBD is poorly understood . The investigators hypothesis is that there are specific clinical, biological, anatomopathological, and radiological presentation specificities of CBD, as well as a worse prognosis compared to pulmonary sarcoidosis.
研究设计
- 研究类型
- Observational
- 观察模型
- Case Control
- 时间视角
- Retrospective
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
- •Age ≥ 18 years
- •Sufficiently documented medical record.
- •Informed patients who did not object to participating in the research, or for deceased patients, did not object to the use of their data.
- •Cases: Patients followed for confirmed chronic beryllium disease by expert teams based on the ATS 2014 criteria, i.e., a history of exposure to beryllium, positivity of two abnormal lymphocyte proliferation tests (LPT) in blood and/or an abnormal LPT in bronchoalveolar lavage, granuloma found in pulmonary biopsy associated by compatible clinical, radiological or spirometric abnormalities.
- •Controls: Patients followed for sarcoidosis according to the ATS/ERS/WASOG criteria, i.e., (i) a compatible presentation, (ii) the presence of non-necrotizing granulomatosis in one or more tissues (except Löfgren's syndrome or Heerfordt syndrome), (iii) and exclusion of alternative causes of granulomatous diseases with pulmonary parenchymal involvement on thoracic CT and/or chest radiography.
- •Controls: without occupational exposure to beryllium.
排除标准
- •Patients under trustee.
结局指标
主要结局
The phenotypic profile at inclusion will be based on clinical data
时间窗: baseline
general signs(number and type of organs affected)
The phenotypic profile at inclusion will be based on radiological data
时间窗: baseline
signs of pulmonary hypertension (absence/presence)
The phenotypic profile at inclusion will be based on biological data
时间窗: baseline
gamma globulinemia (g/L)
The phenotypic profile at inclusion will be based on functional data
时间窗: baseline
6-minute walk test (m)
次要结局
- The occurrence of comorbidities and complications related to the disease and to treatment(baseline)
- Psycho-social consequences(baseline and last visit in 2022)
- Respiratory functional evolution(baseline and last visit in 2022)
- Therapeutic management(baseline)
- Survival without transplantation(From date of baseline until the date of death,or date of lung transplantation or date of last visit whichever came first)
- CT scan evolution(last visit in 2022)