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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.
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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Patients without professional exposure to beryllium | Patients without professional exposure to beryllium | ||
| Patients with chronic pulmonary berylliosis | Patients with chronic pulmonary berylliosis |
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| 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) |
| 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 |
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Inclusion Criteria:
Exclusion Criteria:
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patients with chronic beryllium disease compared to pulmonary sarcoidosis
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Florence JENY, MD | Contact | +331.48.95.52.80 | florence.jeny@aphp.fr | |
| Dominique VALEYRE, MD | Contact | dominique.valeyre@aphp.fr |
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| 001 - Service Pneumologie | Recruiting | Bobigny | Avicenne | France |
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| ID | Term |
|---|---|
| D017565 | Sarcoidosis, Pulmonary |
| ID | Term |
|---|---|
| D017563 | Lung Diseases, Interstitial |
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
| D012507 | Sarcoidosis |
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| 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 |
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 |
| D008232 |
| Lymphoproliferative Disorders |
| D008206 | Lymphatic Diseases |
| D006425 | Hemic and Lymphatic Diseases |
| D006968 | Hypersensitivity, Delayed |
| D006967 | Hypersensitivity |
| D007154 | Immune System Diseases |