Study Comparing Chronic Beryllium Disease to Pulmonary Sarcoidosis

Recruiting

• Conditions: Chronic Beryllium Disease; Pulmonary Sarcoidosis

• Registration Number: NCT06113991
• Lead Sponsor: Assistance Publique - Hôpitaux de Paris

Brief Summary

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.

Detailed Description

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.

Study Timeline

• Status Verified: 2023-05
• Study First Submitted: 2023-06-06
• Study Start: 2023-06-14
• Primary Completion: 2023-06-14
• Study First Submitted QC: 2023-10-27
• Last Update Submitted: 2023-10-27
• Study First Posted: 2023-11-02
• Last Update Posted: 2023-11-02
• Study Completion: 2023-12-14

Recruitment & Eligibility

• Status: RECRUITING
• Sex: All
• Target Recruitment: 200

Inclusion Criteria

• 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.

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Exclusion Criteria

• Patients under trustee.

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Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
The phenotypic profile at inclusion will be based on clinical data	baseline	general signs(number and type of organs affected)
The phenotypic profile at inclusion will be based on biological data	baseline	gamma globulinemia (g/L)
The phenotypic profile at inclusion will be based on functional data	baseline	6-minute walk test (m)
The phenotypic profile at inclusion will be based on radiological data	baseline	signs of pulmonary hypertension (absence/presence)

Secondary Outcome Measures

Name	Time	Method
The occurrence of comorbidities and complications related to the disease and to treatment	baseline	The occurrence of comorbidities will correspond to the presence of comorbidities (smoking, alcoholism, obesity, diabetes, hypertension, other medical history)
Psycho-social consequences	baseline and last visit in 2022	Psycho-social consequences will be evaluated by the number of sick leaves, the existence of professional reclassification, and the number of hospitalizations
Respiratory functional evolution	baseline and last visit in 2022	Respiratory functional evolution will correspond to the absolute variation of the respiratory function parameters extra-functional explorations with measurement in absolute value and as a percentage of the theoretical value of total lung capacity, residual volume, forced vital capacity, maximum exhaled volume, Tiffeneau, carbon monoxide diffusion capacity, transfer coefficient, 6-minute walk test, PaO2, PaCO2
Therapeutic management	baseline	Therapeutic management will be studied by immediate indication of treatment
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	Survival without transplantation will be measured from the date of inclusion until death and/or lung transplantation, or the date of last follow-up.
CT scan evolution	last visit in 2022	CT scan evolution will study the data from the latest available thoracic CT scan

Trial Locations

Locations (1)

001 - Service Pneumologie; 🇫🇷 Bobigny, Avicenne, France