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

Abstract

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 b69) 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.

Justification for screening for chronic beryllium disease: closer to reality

“…In six of the eight patients, the treatment appeared to stop progressive fibrosis. The study by MARCHAND-ADAM et al [19] confirms the earlier report by SEELER [20] and strongly suggests that cases of beryllium disease that could be detected through medical screening programmes would benefit from corticosteroid therapy.…”

“…As a result, corticosteroids and other immunosuppressive medication are considered to be the standard of treatment, and there never have been nor will there ever be controlled trials. The study by MARCHAND-ADAM et al [19], which is the best report of treatment in patients with a confirmed immunological diagnosis of CBD, confirms the earlier report of SEELER [20] and describes computed tomography (CT) findings that appear responsive to corticosteroids. Eight patients with immunologically confirmed CBD were treated with corticosteroids; a 26% improvement was noted in vital capacity and a 15% improvement was seen in diffusion capacity of the lung for carbon monoxide.…”

“…In this issue of the European Respiratory Journal, MARCHAND-ADAM et al [19] address the issue of whether there is a treatment that can prevent or control the disease. The late 1950s' report by SEELER [20], which summarised the beryllium case registry experience with treatment, strongly suggested that corticosteroids were only effective if used early in the disease.…”

Chronic beryllium disease: azathioprine as a possible alternative to corticosteroid treatment

“…Current treatment of CBD relies on both beryllium exposure cessation and corticosteroid therapy. Corticosteroid therapy is efficient on the granulomatous component and leads to a significant clinical, radiological, biological and functional improvement [6]. However, the efficacy of corticosteroids doesn't last: relapses occur following cessation of therapy or dose lowering and long-duration treatment with possible severe side-effects is often necessary.…”

“…However, the efficacy of corticosteroids doesn't last: relapses occur following cessation of therapy or dose lowering and long-duration treatment with possible severe side-effects is often necessary. Eventually, some patients might not respond as expected to corticosteroids, or develop progressive lung fibrosis despite this treatment [6,7].…”