Study Comparing Chronic Beryllium Disease to Pulmonary Sarcoidosis (BERYSARC)

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

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 Overview

• Status: Recruiting
• Conditions: Pulmonary Sarcoidosis; Chronic Beryllium Disease

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 Type: Observational
• Enrollment (Estimated): 200

Contacts and Locations

• Study Contact: Name: Florence JENY, MD; Phone Number: +331.48.95.52.80; Email: florence.jeny@aphp.fr
• Study Contact Backup: Name: Dominique VALEYRE, MD; Email: dominique.valeyre@aphp.fr

Study Locations

• France
  • Avicenne
    • Bobigny, Avicenne, France
      • Recruiting
      • 001 - Service Pneumologie

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Adult; Older Adult
• Accepts Healthy Volunteers: No

• Sampling Method: Non-Probability Sample

Study Population

patients with chronic beryllium disease compared to pulmonary sarcoidosis

Description

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.

Study Plan

How is the study designed?

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort
Patients without professional exposure to beryllium
Patients with chronic pulmonary berylliosis

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
The phenotypic profile at inclusion will be based on clinical data	symptoms at diagnosis	baseline
The phenotypic profile at inclusion will be based on clinical data	general signs(number and type of organs affected)	baseline
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
The phenotypic profile at inclusion will be based on biological data	blood calcium(mmol/L)	baseline
The phenotypic profile at inclusion will be based on biological data	calciuria (mmol/kg/J)	baseline
The phenotypic profile at inclusion will be based on biological data	blood lymphocytes(mm3)	baseline
The phenotypic profile at inclusion will be based on biological data	gamma globulinemia (g/L)	baseline
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
The phenotypic profile at inclusion will be based on functional data	residual volume (%)	baseline
The phenotypic profile at inclusion will be based on functional data	forced vital capacity (L)	baseline
The phenotypic profile at inclusion will be based on functional data	maximum exhaled volume (L)	baseline
The phenotypic profile at inclusion will be based on functional data	Tiffeneau (%)	baseline
The phenotypic profile at inclusion will be based on functional data	carbon monoxide diffusion capacity (%)	baseline
The phenotypic profile at inclusion will be based on functional data	transfer coefficient (%)	baseline
The phenotypic profile at inclusion will be based on functional data	6-minute walk test (m)	baseline
The phenotypic profile at inclusion will be based on radiological data	extent and description on chest CT of elementary lesions activity lesions (mm)	baseline
The phenotypic profile at inclusion will be based on radiological data	fibrosis patterns (absence/presence)	baseline
The phenotypic profile at inclusion will be based on radiological data	signs of pulmonary hypertension (absence/presence)	baseline

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
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
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
Therapeutic management	Therapeutic management will be studied by immediate indication of treatment	baseline
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
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
CT scan evolution	CT scan evolution will study the data from the latest available thoracic CT scan	last visit in 2022

Collaborators and Investigators

• Sponsor: Assistance Publique - Hôpitaux de Paris

Study record dates

Study Major Dates

• Study Start (Actual): June 14, 2023
• Primary Completion (Actual): June 14, 2023
• Study Completion (Estimated): December 14, 2023

Study Registration Dates

• First Submitted: June 6, 2023
• First Submitted That Met QC Criteria: October 27, 2023
• First Posted (Actual): November 2, 2023

Study Record Updates

• Last Update Posted (Actual): November 2, 2023
• Last Update Submitted That Met QC Criteria: October 27, 2023
• Last Verified: May 1, 2023

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Respiratory Tract Diseases; Immune System Diseases; Lymphoproliferative Disorders; Lymphatic Diseases; Lung Diseases; Occupational Diseases; Hypersensitivity; Pneumoconiosis; Lung Diseases, Interstitial; Lung Injury; Hypersensitivity, Delayed; Sarcoidosis, Pulmonary; Sarcoidosis; Berylliosis
• Other Study ID Numbers: APHP230386

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No