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.
Boehler
A
A
Dept of Internal Medicine, University Hospital, Zuerich, Switzerland.
Vogt
P
P
Speich
R
R
Weder
W
W
Russi
E W
EW
D018501:Antirheumatic Agents / Q000009:adverse effects* / Q000627:therapeutic use; D001989:Bronchiolitis Obliterans / Q000139:chemically induced* / Q000473:pathology / Q000601:surgery; D006801:Humans; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D010396:Penicillamine / Q000009:adverse effects* / Q000627:therapeutic use; D012129:Respiratory Function Tests; D012595:Scleroderma, Systemic / Q000188:drug therapy
D018501:Antirheumatic Agents; D010396:Penicillamine