Study Comparing Chronic Beryllium Disease to Pulmonary Sarcoidosis

Pulmonary Sarcoidosis Clinical Trial

— BERYSARC  

Official title:

Retrospective Multicenter Case-control Study Comparing Chronic Beryllium Disease to Pulmonary Sarcoidosis

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT06113991
• Other study ID #: APHP230386
• Status: Recruiting
• Start date: June 14, 2023
• Est. completion date: December 14, 2023

Study information

• Verified date: May 2023
• Source: Assistance Publique - Hôpitaux de Paris
• Contact: Florence JENY, MD
• Phone: +331.48.95.52.80
• Email: florence.jeny[@]aphp.fr
• Is FDA regulated: No
• Study type: Observational

Clinical Trial 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.

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.

Recruitment information / eligibility

• Status: Recruiting
• Enrollment: 200
• Est. completion date: December 14, 2023
• Est. primary completion date: June 14, 2023
• Accepts healthy volunteers: No
• Gender: All
• Age group: 18 Years and older

Eligibility

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.

Exclusion Criteria:

• Patients under trustee.

Related Conditions & MeSH terms

• Berylliosis
• Chronic Beryllium Disease
• Pulmonary Sarcoidosis
• Sarcoidosis
• Sarcoidosis, Pulmonary

Locations

Country	Name	City	State
France	001 - Service Pneumologie	Bobigny	Avicenne

Sponsors (1)

Lead Sponsor	Collaborator
Assistance Publique - Hôpitaux de Paris

Country where clinical trial is conducted

France, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	The phenotypic profile at inclusion will be based on clinical data	symptoms at diagnosis	baseline
Primary	The phenotypic profile at inclusion will be based on clinical data	general signs(number and type of organs affected)	baseline
Primary	The phenotypic profile at inclusion will be based on biological data	serum angiotensin-converting enzyme assay (hydrolysis of one micromole of substrate per minute)	baseline
Primary	The phenotypic profile at inclusion will be based on biological data	blood calcium(mmol/L)	baseline
Primary	The phenotypic profile at inclusion will be based on biological data	calciuria (mmol/kg/J)	baseline
Primary	The phenotypic profile at inclusion will be based on biological data	blood lymphocytes(mm3)	baseline
Primary	The phenotypic profile at inclusion will be based on biological data	gamma globulinemia (g/L)	baseline
Primary	The phenotypic profile at inclusion will be based on functional data	extra-functional explorations with measurement in absolute value and as a percentage of the theoretical value of total lung capacity (L)	baseline
Primary	The phenotypic profile at inclusion will be based on functional data	residual volume (%)	baseline
Primary	The phenotypic profile at inclusion will be based on functional data	forced vital capacity (L)	baseline
Primary	The phenotypic profile at inclusion will be based on functional data	maximum exhaled volume (L)	baseline
Primary	The phenotypic profile at inclusion will be based on functional data	Tiffeneau (%)	baseline
Primary	The phenotypic profile at inclusion will be based on functional data	carbon monoxide diffusion capacity (%)	baseline
Primary	The phenotypic profile at inclusion will be based on functional data	transfer coefficient (%)	baseline
Primary	The phenotypic profile at inclusion will be based on functional data	6-minute walk test (m)	baseline
Primary	The phenotypic profile at inclusion will be based on radiological data	extent and description on chest CT of elementary lesions activity lesions (mm)	baseline
Primary	The phenotypic profile at inclusion will be based on radiological data	fibrosis patterns (absence/presence)	baseline
Primary	The phenotypic profile at inclusion will be based on radiological data	signs of pulmonary hypertension (absence/presence)	baseline
Secondary	Survival without transplantation	Survival without transplantation will be measured from the date of inclusion until death and/or lung transplantation, or the date of last follow-up.	From date of baseline until the date of death,or date of lung transplantation or date of last visit whichever came first
Secondary	The occurrence of comorbidities and complications related to the disease and to treatment	The occurrence of comorbidities will correspond to the presence of comorbidities (smoking, alcoholism, obesity, diabetes, hypertension, other medical history)	baseline
Secondary	Therapeutic management	Therapeutic management will be studied by immediate indication of treatment	baseline
Secondary	Psycho-social consequences	Psycho-social consequences will be evaluated by the number of sick leaves, the existence of professional reclassification, and the number of hospitalizations	baseline and last visit in 2022
Secondary	Respiratory functional evolution	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	baseline and last visit in 2022
Secondary	CT scan evolution	CT scan evolution will study the data from the latest available thoracic CT scan	last visit in 2022