Epidemiology

• Indications
  • Connective Tissue Diseases

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Clinical Patterns

Pulmonary Manifestations

• Bronchiolitis Obliterans (see Bronchiolitis Obliterans)
  • Clinical: usually present with already advanced BO
  • Treatment: corticosteroids have little benefit
• Drug-Induced Systemic Lupus Erythematosus (see Systemic Lupus Erythematosus)
  • Epidemiology: penicillamine is one of the more common causes of drug-induced SLE
  • Diagnosis
    • CXR/Chest CT: pleural effusion (with normal pleural glucose)
• Penicillamine-Induced Goodpasture’s Syndrome (see Goodpasture’s Syndrome)
  • Diagnosis
    • CXR/Chest CT: diffuse or patchy alveolar infiltrates (interstitial infiltrates may occur with recurrent alveolar hemorrhage)
    • PFT’s: decreased DLCO during alveolar hemorrhage
  • Clinical: diffuse alveolar hemorrhage
• Diffuse Alveolar Hemorrhage (see Diffuse Alveolar Hemorrhage): only 8 reported cases of DAH in patients being treated for RA, PBC, and Wilson’s disease
  • Epidemiology: cases have occurred with >1 g/day for at least 10 months (some cases have occurred after 20 years of daily treatment)
  • Pathology: absence of pulmonary capillaritis, hemosiderin-laden macrophages
  • Diagnosis
    • CXR/Chest CT: diffuse or patchy alveolar infiltrates (interstitial infiltrates may occur with recurrent alveolar hemorrhage)
    • PFT’s: decreased DLCO during alveolar hemorrhage
  • Clinical: cough, dyspnea, hemoptysis (may be absent on initial presentation in some cases, even after significant bleed), chest pain
• Cryptogenic Organizing Pneumonia (see Cryptogenic Organizing Pneumonia)
• Pneumonia-Like Presentation (see Pneumonia): this likely represents unrecognized drug-induced SLE
• Scleroderma-Like Syndrome (see Scleroderma):

Renal Manifestations

• Nephrotic Syndrome

Other Manifestations

• Absence of Rheum or Derm Involvement

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Treatment

• Immunosuppressives + Plasmapheresis: effective in a few cases

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References

• D-penicillamine-induced myasthenia gravis in a case of eosinophilic fasciitis. J Neurol Sci 1997; 146: 85-86
• Organizing pneumonia. Features and prognosis of cryptogenic, secondary, and focal variants. Arch Intern Med 1997; 157: 1323-1329
• Organizing diffuse alveolar damage associated with progressive systemic sclerosis. Mayo Clin Proc 1997; 72: 639-642
• Bronchiolitis obliterans in a patient with localized scleroderma treated with D-penicillamine. Eur Respir J 1996; 9: 1317-1319
• Pleural effusion associated with d-penicillamine therapy: a case report. Journal of Clinical Pharmacology and Therapeutics 1996; 21: 15-17
• D-penicillamine-induced myasthenia gravis: diagnosis obscured by coexisting chronic obstructive pulmonary disease. Am J Med Sci 1995; 309: 191-193
• Non-fatal bronchiolitis obliterans possibly associated with tiopronin. A case report with long term follow-up. Br J Rheumatol 1993; 32: 172-174
• A case of rheumatoid arthritis with obstructive bronchiolitis appearing after d-penicillamine therapy. Nippon Kyobu Shikkan Gakkai Zasshi 1993; 31: 1195-1200
• High-resolution computed tomography of drug-induced lung disease. Clin Radiol 1992; 46: 232-236
• Drug-induced pulmonary disease: an update. Chest 1992; 102: 239-250
• Pulmonary infiltration and eosinophilia associated with sulfasalazine therapy for ulcerative colitis: a case report and review of the literature. Intern Med 1992; 31: 108-113
• Bronchoalveolar lavage cell data in 19 patients with drug-associated pneumonitis (except amiodarone). Chest 1991; 99: 98-104
• Rheumatoid arthritis with extensive lung lesions. Thorax 1989; 44: 70-71
• Adverse effects of d-penicillamine. A cooperative study by the french regional drug surveillance centers. J Rheumatol 1988; 15: 1730-1732
• D-penicillamine-induced acute hypersensitivity pneumonitis and cholestatic hepatitis in a patient with rheumatoid arthritis. Clin Exp Rheumatol 1985; 3: 337-339
• Beneficial effect of intravenous cyclophosphamide and oral prednisone on d-penicillamine-associated bronchiolitis obliterans. Arthr Rheum 1985; 28: 93-97
• Lung biopsy in rheumatoid arthritis. Am Rev Respir Dis 1985; 131: 770-777
• Bronchiolitis in rheumatoid arthritis. Chest 1984; 85: 705-708
• Upper and lower airway disease in penicillamine treated patients with rheumatoid arthritis. J Rheumatol 1983; 10: 406-410
• Airway disease in a subset of nonsmoking rheumatoid patients. Am J Med 1982; 72: 743-750
• D-penicillamine-induced severe pneumonitis. Chest 1982; 81: 376-378
• D-penicillamine therapy in progressive systemic sclerosis (scleroderma). A retrospective analysis. Ann Intern Med 1982; 97: 652-659
• Relationship of gold and penicillamine therapy to diffuse interstitial lung disease. Ann Rheum Dis 1981; 40: 136-141
• Cryptogenic obliterative bronchiolitis in adults. Thorax 1981; 36: 805-810
• Pulmonary eosinophilia caused by penicillamine. Thorax 1980; 35: 957-958
• D-penicillamine-induced bronchial spasm. South Med J 1980; 73: 788-790
• Bronchiolitis and bronchitis in connective tissue disease. A possible relationship to the use of penicillamine. JAMA 1979; 242: 528-532
• Iatrogenic pulmonary pathology in patients with rheumatoid arthritis against the background of d-penicillamine treatment. Sov Med 1979; 10: 9-11
• Miliary pulmonary infiltrates and penicillamine. Br J Radiol 1978; 51: 915-916
• Diffuse alveolitis as complication of penicillamine treatment for rheumatoid arthritis. Br Med J 1976; i: 1506
• D-penicillamine induced Goodpasture’s syndrome in Wilson’s disease. Ann Intern Med 1975; 82: 673-676