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Logo of procrsmedFormerly medchtJournal of the Royal Society of MedicineProceedings of the Royal Society of Medicine
Proc R Soc Med. 1977; 70(Suppl 3): 82–88.
PMCID: PMC1543570

Penicillamine therapy in systemic sclerosis.


Twenty-two patients with progressive systemic sclerosis were treated with D-penicillamine in doses ranging up to 1250 mg/day for periods varying between a few months and four years. Side-effects occurred in 7 patients, necessitating drug withdrawal in 2. Cutaneous benefit occurred in 15 patients, but owing to side-effects from the drug, relapses, and development, persistence or advancement of visceral complications, an overall good result only occurred in 5. Seven patients showed improvements in joint function, but only 3 were regarded as having an overall good result. Peripheral vascular disease and visceral involvement seemed not to be influenced by D-penicillamine and sometimes appeared or advanced during treatment. Six patients died from visceral manifestations of systemic sclerosis and one from another cause. D-penicillamine is of limited value for the cutaneous features of progressive systemic sclerosis, but probably of no value for the vascular and visceral manifestations of the disease.

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Selected References

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  • Alarcón-Segovia D, Ibáez G, Kershenobich D, Rojkind M. Letter: Treatment of scleroderma. Lancet. 1974 May 25;1(7865):1054–1055. [PubMed]
  • Bailey AJ, Robins SP, Balian G. Biological significance of the intermolecular crosslinks of collagen. Nature. 1974 Sep 13;251(5471):105–109. [PubMed]
  • Bennett R, Bluestone R, Holt PJ, Bywaters EG. Survival in scleroderma. Ann Rheum Dis. 1971 Nov;30(6):581–588. [PubMed]
  • 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 Mar;29(2):153–158. [PMC free article] [PubMed]
  • Borisy GG, Taylor EW. The mechanism of action of colchicine. Binding of colchincine-3H to cellular protein. J Cell Biol. 1967 Aug;34(2):525–533. [PMC free article] [PubMed]
  • Campbell PM, LeRoy EC. Pathogenesis of systemic sclerosis: a vascular hypothesis. Semin Arthritis Rheum. 1975 May;4(4):351–368. [PubMed]
  • Chvapil M. Pharmacology of fibrosis: definitions, limits and perspectives. Life Sci. 1975 May 1;16(9):1345–1361. [PubMed]
  • Chvapil M, Madden JW, Carlson EC, Peacock EE., Jr Effect of cis-hydroxyproline on collagen and other proteins in skin wounds, granuloma tissue, and liver of mice and rats. Exp Mol Pathol. 1974 Jun;20(3):363–373. [PubMed]
  • Dabich L, Bookstein JJ, Zweifler A, Zarafonetis CJ. Digital arteries in patients with scleroderma. Arteriographic and plethysmographic study. Arch Intern Med. 1972 Nov;130(5):708–714. [PubMed]
  • D'Angelo WA, Fries JF, Masi AT, Shulman LE. Pathologic observations in systemic sclerosis (scleroderma). A study of fifty-eight autopsy cases and fifty-eight matched controls. Am J Med. 1969 Mar;46(3):428–440. [PubMed]
  • Ehrlich HP, Ross R, Bornstein P. Effects of antimicrotubular agents on the secretion of collagen. A biochemical and morphological study. J Cell Biol. 1974 Aug;62(2):390–405. [PMC free article] [PubMed]
  • Fleischmajer R, Damiano V, Nedwich A. Alteration of subcutaneous tissue in systemic scleroderma. Arch Dermatol. 1972 Jan;105(1):59–66. [PubMed]
  • Fulghum DD, Katz R. Penicillamine for scleroderma. Arch Dermatol. 1968 Jul;98(1):51–52. [PubMed]
  • Hayes RL, Rodnan GP. The ultrastructure of skin in progressive systemic sclerosis (scleroderma). I. Dermal collagen fibers. Am J Pathol. 1971 Jun;63(3):433–442. [PubMed]
  • Herbert CM, Lindberg KA, Jayson MI, Bailey AJ. Biosynthesis and maturation of skin collagen in scleroderma, and effect of D-penicillamine. Lancet. 1974 Feb 9;1(7850):187–192. [PubMed]
  • Keiser HR, Stein HD, Sjoerdsma A. Increased protocollagen proline hydroxylase activity in sclerodermatous skin. Arch Dermatol. 1971 Jul;104(1):57–60. [PubMed]
  • Leroy EC. Connective tissue synthesis by scleroderma skin fibroblasts in cell culture. J Exp Med. 1972 Jun 1;135(6):1351–1362. [PMC free article] [PubMed]
  • Madden JW, Chvapil M, Carlson EC, Ryan JN. Toxicity and metabolic effects of 3,4-dehydroproline in mice. Toxicol Appl Pharmacol. 1973 Nov;26(3):426–437. [PubMed]
  • Maricq HR, LeRoy EC. Patterns of finger capillary abnormalities in connective tissue disease by "wide-field" microscopy. Arthritis Rheum. 1973 Sep-Oct;16(5):619–628. [PubMed]
  • Moynahan EJ. Morphoea (localized cutaneous scleroderma) treated with low-dosage penicillamine (4 cases, including coup de sabre). Proc R Soc Med. 1973 Nov;66(11):1083–1085. [PMC free article] [PubMed]
  • Nimni ME, Deshmukh K, Gerth N. Collagen defect induced by penicillamine. Nat New Biol. 1972 Dec 13;240(102):220–221. [PubMed]
  • Norton WL, Hurd ER, Lewis DC, Ziff M. Evidence of microvascular injury in scleroderma and systemic lupus erythematosus: quantitative study of the microvascular bed. J Lab Clin Med. 1968 Jun;71(6):919–933. [PubMed]
  • BLAKEMORE WS, FORSTER RE, MORTON JW, OGILVIE CM. A standardized breath holding technique for the clinical measurement of the diffusing capacity of the lung for carbon monoxide. J Clin Invest. 1957 Jan;36(1 Pt 1):1–17. [PMC free article] [PubMed]
  • Robins SP, Shimokomaki M, Bailey AJ. The chemistry of the collagen cross-links. Age-related changes in the reducible components of intact bovine collagen fibres. Biochem J. 1973 Apr;131(4):771–780. [PubMed]
  • Uitto J, Halme J, Hannuksela M, Peltokallio P, Kivirikko KI. Protocollagen proline hydroxylase activity in the skin of normal human subjects and of patients with scleroderma. Scand J Clin Lab Invest. 1969 May;23(3):241–247. [PubMed]

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