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

Eur Respir J. 1996 Jun;9(6):1317-9. doi: 10.1183/09031936.96.09061317.


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.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Antirheumatic Agents / adverse effects*
  • Antirheumatic Agents / therapeutic use
  • Bronchiolitis Obliterans / chemically induced*
  • Bronchiolitis Obliterans / pathology
  • Bronchiolitis Obliterans / surgery
  • Humans
  • Lung Transplantation
  • Male
  • Middle Aged
  • Penicillamine / adverse effects*
  • Penicillamine / therapeutic use
  • Respiratory Function Tests
  • Scleroderma, Systemic / drug therapy


  • Antirheumatic Agents
  • Penicillamine