Chronic beryllium disease
Chronic beryllium disease is a noncaseating granulomatous lung disease caused by inhaled beryllium in aerospace, nuclear, electronics, ceramics, and dental-material workers, and it mimics sarcoidosis clinically and histologically. The beryllium lymphocyte proliferation test is the initial and diagnostic test. Treatment is removal from exposure plus chronic glucocorticoids, since it is the one interstitial lung disease that reliably responds to steroids.
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What this article covers
- Overview
Definition: Chronic beryllium disease (CBD, berylliosis) is a granulomatous interstitial lung disease caused by inhaling beryllium at work, producing noncaseating granulomas that favor the upper lobes.
- Epidemiology
Typical patient: A worker in aerospace, automotive, nuclear (including nuclear weapons), telecommunications, or electronics manufacturing who handles beryllium-containing material.
- Risk factors
Beryllium handling at work: The defining risk; disease does not occur without exposure, which is why the occupational history is the single most important step.
- Pathophysiology
Antigen presentation: Inhaled beryllium bound to human leukocyte antigen (HLA)-encoded major histocompatibility complexes strongly engages CD4+ T-cell receptors.
- Clinical features
Hallmark presentation: Progressive exertional dyspnea and a nonproductive cough in a beryllium-exposed worker; symptoms may ease away from work and return on going back.
- Differential diagnosis
Sarcoidosis: The most important mimic, with identical noncaseating granulomas and hilar adenopathy, but no beryllium exposure, a negative BeLPT, and frequent extrapulmonary disease such as ocular inflammation.
- Diagnosis
What establishes the diagnosis: CBD is confirmed by a positive BeLPT in a patient with documented beryllium exposure and a granulomatous lung disease (compatible upper-lobe infiltrates with hilar…
- Management
First action: Remove the worker from all further beryllium exposure, and investigate the workplace so every other affected employee is identified.
- Complications
Progressive pulmonary fibrosis and respiratory failure: The end result of untreated or continued-exposure granulomatous inflammation.
- Prognosis
Steroid-responsive disease: CBD is the interstitial lung disease most reliably improved by glucocorticoids, giving it a better outlook than the fibrotic pneumoconioses for which no therapy exists.
- Prevention
Exposure elimination: Removal of the worker from beryllium, or of beryllium from the workplace, is the primary and only disease-specific preventive measure.
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