Skip to main content
SpringerLink
Log in

Penicillamine in the treatment of systemic sclerosis

  • Published:
Current Rheumatology Reports Aims and scope Submit manuscript

Abstract

Retrospective studies have suggested that D-penicillamine (DPA) in conventional high dosages (750-1000 mg/d) may be efficacious in treating the skin and visceral complications of systemic sclerosis (SSc), particularly when used early in the disease. The course of skin thickening and the occurrence of renal crisis and of death were examined in a recent randomized controlled 2-year trial of highdosage DPA (750-1000 mg/d) versus low-dose DPA (125 mg every other day). Skin thickening improved in both groups to a similar extent, and the occurrence of renal crisis and of death were not different in the two groups. Although the trial was not designed to answer the question of whether low-dosage DPA is effective, it did suggest that there is no advantage to using DPA in doses higher than 125 mg every other day.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References and Recommended Reading

  1. Harris ED, Sjoerdsma A: Effect of penicillamine on human collagen and possible application to treatment of scleroderma. Lancet 1966, 2:996–999.

    Article  PubMed  Google Scholar 

  2. Fulghum DD, Katz R: Penicillamine for scleroderma. Arch Dermatol 1968, 98:51–52.

    Article  PubMed  CAS  Google Scholar 

  3. Bluestone R, Grahame R, Holloway V, Holt PJ: Treatment of systemic sclerosis with D-penicillamine. A new method of observing the effects of treatment. Ann Rheum Dis 1970, 29:153–158.

    Article  PubMed  CAS  Google Scholar 

  4. Winkelmann RK, Kierland RR, Perry HD: Management of scleroderma. Mayo Clin Proc 1971, 46:128–134.

    PubMed  CAS  Google Scholar 

  5. Lorenzen IB, Uitto J, Ohlenschlaeger K: D-DPA in progressive scleroderma. Scand J Rheumatol 1972, 1:121–124.

    PubMed  CAS  Google Scholar 

  6. Tio H, van Wikk L, Haan E: Treatment of progressive systemic sclerosis (PSS) with penicillamine. Preliminary report of two cases. Acta Med Scand 1973, 193:477–480.

    PubMed  CAS  Google Scholar 

  7. Herbert CM, Lindberg KA, Jayson MI, Bailey AJ: Biosynthesis and maturation of skin collagen in scleroderma, and effect of D-penicillamine. Lancet 1974, 1:187–192.

    Article  PubMed  CAS  Google Scholar 

  8. Asboe-Hansen G: Treatment of generalized scleroderma with inhibitors of connective tissue formation. Acta Derm Venereol (Stockh) 1975, 55:461–465.

    CAS  Google Scholar 

  9. Zachariae H, Petersen HO, Zachariae E: D-penicillamine in scleroderma: a preliminary study. In Penicillamine Research in Rheumatoid Disease. Edited by Munthe E. Oslo, Norway: Fabritius and Sonner; 1976: 290.

    Google Scholar 

  10. Jayson MIV, Lovell C, Black CM, Wilson RS: Penicillamine therapy in systemic sclerosis. Proc R Soc Med 1977, 70:82–88.

    PubMed  Google Scholar 

  11. Mellstedt H, Fagrell B, Bjokholm M: Effect on nutritional capillary circulation. Scand J Rheumatol 1977, 6:92–96.

    PubMed  CAS  Google Scholar 

  12. Steen VD, Medsger TA, Jr, Rodnan GP: D-penicillamine therapy in progressive systemic sclerosis (scleroderma): a retrospective analysis. Ann Intern Med 1982, 97:652–659.

    PubMed  CAS  Google Scholar 

  13. Jimenez SA, Sigal SH: A 15-year prospective study of treatment of rapidly progressive systemic sclerosis with D-penicillamine. J Rheum 1991, 18:1496–1503.

    PubMed  CAS  Google Scholar 

  14. Steen VD, Blair S, Medsger TA Jr.: The toxicity of D-DPA in systemic sclerosis. Ann Intern Med 1986, 104:699–705. Seminal study which propelled the interest in using D-penicillamine in systemic sclerosis. It provided preliminary data for design of a randomized controlled trial - the High-Dose vs Low-Dose Penicillamine in Early Diffuse Systemic Sclerosis Trial.

    PubMed  CAS  Google Scholar 

  15. Clements PJ, Wong WK, Seibold JR, et al.: High-dose (HI-DPA) vs low-dose (LO-DPA) penicillamine in early diffuse systemic sclerosis (SSc) trial: analysis of trial. Arthritis Rheum. 1997, 40:S173. Both references provide the results of the analysis of the High-Dose vs Low-Dose Penicillamine in Early Diffuse Systemic Sclerosis Trial, which showed that the skin scores in both groups improved to the same degree and that there were no significant differences in the occurrence of renal crisis or death between the two treatment groups.

    Google Scholar 

  16. Clements PJ, Wong WK, Seibold JR, et al.: High-dose (HI-DPA) vs low-dose (LO-DPA) penicillamine in early diffuse systemic sclerosis (SSc) trial: analysis of trial. Arthritis Rheum, in press. Both references provide the results of the analysis of the High-Dose vs Low-Dose Penicillamine in Early Diffuse Systemic Sclerosis Trial, which showed that the skin scores in both groups improved to the same degree and that there were no significant differences in the occurrence of renal crisis or death between the two treatment groups.

  17. Medsger TA, Jr, Steen VD: Classification, prognosis. In Systemic Sclerosis. Edited by Clements PJ, Furst DE. Baltimore: Williams and Wilkins; 1996: 51–64.

    Google Scholar 

  18. White B, Bauer EA, Goldsmith LA, et al.: Guidelines for clinical trials in systemic sclerosis (scleroderma). I. Diseasemodifying interventions. Arthritis Rheum 1995, 38:351–360.

    Article  PubMed  CAS  Google Scholar 

  19. Colditz GA, Miller JN, Mosteller F: How study design affects outcomes in comparisons of therapy. I: Medical. Stat Med 1989, 8:441–444.

    Article  CAS  Google Scholar 

  20. Jadad AR, Moore RA, Carroll D, et al.: Assessing the quality of report of randomized clinical trials: is blinding necessary? Control Clin Trials 1996, 17:1–12.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Medicine, Division of Rheumatology, University of California at Los Angeles, School of Medicine, Los Angeles, California

    Philip J. Clements MD, MPH

Authors
  1. Philip J. Clements MD, MPH
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Clements, P.J. Penicillamine in the treatment of systemic sclerosis. Curr Rheumatol Rep 1, 38–42 (1999). https://doi.org/10.1007/s11926-999-0023-3

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11926-999-0023-3

Keywords

Search

Navigation