Berylliosis

Berylliosis (chronic beryllium disease, CBD) is a granulomatous lung disease caused by a cell mediated hypersensitivity (type IV) to beryllium, mimicking sarcoidosis.

• Exposure: aerospace, nuclear, electronics, ceramics, dental alloys, metal machining and recycling, jewellery; even low level exposure; genetic susceptibility (HLA DPB1 Glu69 in 90%).
• Beryllium sensitisation (positive lymphocyte proliferation test) occurs in about 1 to 16% of exposed workers; progression to CBD in 3 to 5%.
• Acute beryllium disease (chemical pneumonitis) with high dose exposure is rare today.
• Latency months to decades; progression to fibrosis, respiratory failure; lung cancer association (IARC group 1).

• Exertional dyspnoea, dry cough, fatigue, chest pain, weight loss, arthralgia, night sweats.
• Crackles, clubbing, skin granulomas, erythema nodosum; extrapulmonary granulomas (liver, nodes, skin).
• Distinguish from sarcoidosis through exposure history and BeLPT.

• Occupational history of beryllium exposure.
• Beryllium lymphocyte proliferation test (BeLPT) on blood and bronchoalveolar lavage cells: two abnormal blood results or one abnormal BAL result confirms sensitisation.
• CBD diagnosis: sensitisation with granulomas on lung biopsy (transbronchial) or BAL lymphocytosis (over 20%) with an abnormal BeLPT.
• HRCT: small nodules, ground glass, septal thickening, hilar lymphadenopathy, fibrosis; chest radiograph.
• Pulmonary function: restrictive, obstructive or mixed; reduced DLCO; exercise gas exchange abnormalities.
• Medical surveillance of workers (BeLPT screening).

• Remove from beryllium exposure (cornerstone) and report; sensitised workers require surveillance every 1 to 3 years.
• Corticosteroids for symptomatic CBD or progressive physiological decline: prednisolone 20 to 40 mg daily for 1 to 3 months, taper to lowest effective dose (5 to 10 mg) for 12 months or more; response rates variable.
• Steroid sparing: methotrexate 10 to 20 mg weekly, azathioprine 2 mg/kg, mycophenolate; infliximab for refractory disease.
• Supportive: bronchodilators, oxygen, pulmonary rehabilitation, vaccinations, smoking cessation.
• Monitor spirometry, DLCO and HRCT every 6 to 12 months; bone protection and Pneumocystis prophylaxis during immunosuppression.
• Lung transplantation for end stage; lung cancer screening.
• Exposure controls: engineering controls, respirators, occupational limits, compensation.