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Short- and long-term response to corticosteroid therapy in chronic beryllium disease

¹ Université Paris 13, EA2363, Assistance Publique-HÂpital de Pol's, Service de Pneumologie, Hôpital Universitaire Avicenne, Bobigny, ² INSERM U618, Université François-Rabelais, Tours, and ³ Assistance Publique-Hôpitaux de Paris and INSERM U662, Hôpital Saint-Louis, Paris, France.

CORRESPONDENCE: D. Valeyre, Service de Pneumologie, Hôpital Avicenne, 125 rue de Stalingrad, 93009 Bobigny Cedex, France. Fax: 33 148955126 E-mail: dominique.valeyre@avc.aphp.fr

Keywords: Berylliosis, chronic beryllium disease, corticosteroids, Glu⁶⁹, outcome, sarcoidosis

Received: November 8, 2007
Accepted May 23, 2008

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, Glu⁶⁹ (HLA-DPB1 genes coding for glutamate at position β69) 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.