Penicillamine

Abstract

S Penicillamine Bronchiolitis obliterans: case reportA 47-year-old man developed bronchiolitis obliterans during treatment with penicillamine for hepatolenticular degeneration (Wilson's disease).The man had a history of hypertension, dyslipidaemia treated with statins, Graves-Basedow disease treated with replacement therapy; he also had Wilson's disease, diagnosed 20 years earlier, for which he had received penicillamine for the previous 2 years [dosage and route not stated]. He presented with dyspnoea on exertion and an initial lung function study showed severe airflow limitation.Inhaled corticosteroids were initiated. Complete lung function testing showed severe irreversible obstructive changes with air trapping and reduced carbon monoxide diffusing capacity. A chest CT scan revealed discrete bronchiectasis and diffuse bronchiolectasis. A sweat test excluded cystic fibrosis and an immunoglobulin study was normal. Respiratory failure was observed during a follow-up visit and the man was admitted for further tests. Examination revealed an oxygen saturation of 88% on room air, without laboured breathing, and normal pulmonary auscultation. As drug-induced bronchiolitis was suspected, a lingular biopsy was obtained via anterior thoracotomy. He was subsequently discharged with domiciliary oxygen. The final diagnosis from the pathological study was follicular bronchiolitis associated with constrictive bronchiolitis obliterans. Penicillamine was withdrawn and he began receiving bronchodilators and methylprednisolone. He subsequently underwent a successful double lung transplant.Author comment: "To conclude, this clinical case is presented as an example of an infrequent but previously described side effect of a treatment [penicillamine] which is commonly used in several diseases." Bruguera-Avila N, et al. Obliterating bronchiolitis in a patient treated with (D)penicillamine. Archivos de Bronconeumologia 49: 411-2, No. 9,