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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)

Bronchiolitis obliterans in a patient with localized scleroderma treated with D-penicillamine.

D-penicillamine-associated bronchiolitis obliterans (BO) is a rare but well-known pulmonary complication in patients with rheumatoid arthritis or progressive systemic sclerosis. It has been assumed that in most, if not all cases, BO is a complication of the underlying disease rather than a side-effect of treatment. We report the case of a 46 year old man with scleroderma localized to his lower legs (morphea), who received a daily dose of 750 mg D-penicillamine. During the treatment of 1 yr duration, he developed progressive shortness of breath due to a worsening obstructive ventilatory defect suggesting BO, which was confirmed by surgical lung biopsy (constrictive BO). Bronchial obstruction progressed over the next 5 yrs and did not respond to corticosteroids. The patient finally underwent a successful single left lung transplantation. The histological features of constrictive BO were confirmed in the explanted lung. This observation suggests that D-penicillamine may induce bronchiolitis obliterans in the absence of a systemic connective tissue disease.[1]


  1. Bronchiolitis obliterans in a patient with localized scleroderma treated with D-penicillamine. Boehler, A., Vogt, P., Speich, R., Weder, W., Russi, E.W. Eur. Respir. J. (1996) [Pubmed]
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