Chronic
beryllium disease (CBD) is a granulomatous disorder that affects the lung after exposure to
beryllium. The present study reports short- and long-term evolution of granulomatous and fibrotic components in eight patients with severe CBD receiving
corticosteroid therapy. Eight patients with confirmed CBD were studied at baseline, after initial
corticosteroid treatment (4-12 months), at relapse and at the final visit.
Beryllium exposure, Glu(69) (
HLA-DPB1 genes coding for
glutamate at position beta69) polymorphism, symptoms, pulmonary function tests (PFT), serum
angiotensin-converting enzyme (SACE) and high-resolution computed tomography (HRCT) quantification of pulmonary lesions were analysed. The CBD patients were observed for a median (range) of 69 (20-180) months. After stopping
beryllium exposure,
corticosteroids improved symptoms and PFT (vital capacity +26%, diffusing capacity of the lung for
carbon monoxide +15%), and decreased SACE level and active lesion HRCT score. In total, 18 clinical relapses occurred after the treatment was tapered and these were associated with SACE and active lesion HRCT score impairment. At the final visit,
corticosteroids had completely stabilised all parameters including both HRCT scores of active lesions and fibrotic lesions in six out of eight patients.
Corticosteroids were beneficial in chronic
beryllium disease. They were effective in suppressing granulomatosis lesions in all cases and in stopping the evolution to
pulmonary fibrosis in six out of eight patients.