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Principles of Management

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Scleroderma
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Abstract

The care of a patient with systemic sclerosis (scleroderma) is particularly challenging because of the multiple potential complications of scleroderma, the variable clinical course, and imperfect treatment options. For many rheumatologists, as well as for our patients, this situation has resulted in a pessimistic attitude toward therapy that may not be warranted. It is clear that the management of scleroderma requires a multidisciplinary approach, and the responsibility to integrate results and coordinate care usually falls to the rheumatologist. From the patients’ perspective, visits to multiple subspecialties can lead to confusing or even conflicting advice and inadequate explanations as to the cause of their symptoms.

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References

  1. Steen VD. The many faces of scleroderma. Rheum Dis Clin North Am. 2008;34(1):1–15 v. PMID: 18329529.

    Article  PubMed  Google Scholar 

  2. LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger Jr TA, Rowell N, Wollheim F. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol. 1988;15(2):202–5. PMID: 3361530.

    PubMed  CAS  Google Scholar 

  3. Khanna D, Denton CP. Evidence-based management of rapidly progressing systemic sclerosis. Best Pract Res Clin Rheumatol. 2010;24(3):387–400. Review. PMID: 20534372.

    Article  PubMed  Google Scholar 

  4. Steen VD. Autoantibodies in systemic sclerosis. Semin Arthritis Rheum. 2005;35(1):35–42. PMID: 16084222.

    Article  PubMed  CAS  Google Scholar 

  5. Tashkin DP, Elashoff R, Clements PJ, Goldin J, Roth MD, Furst DE, Arriola E, Silver R, Strange C, Bolster M, Seibold JR, Riley DJ, Hsu VM, Varga J, Schraufnagel DE, Theodore A, Simms R, Wise R, Wigley F, White B, Steen V, Read C, Mayes M, Parsley E, Mubarak K, Connolly MK, Golden J, Olman M, Fessler B, Rothfield N, Metersky M. Scleroderma lung study research group. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med. 2006;354(25):2655–66. PMID: 16790698.

    Article  PubMed  CAS  Google Scholar 

  6. Henness S, Wigley FM. Current drug therapy for scleroderma and secondary Raynaud’s phenomenon: evidence-based review. Curr Opin Rheumatol. 2007;19(6):611–8. Review.PMID: 17917543.

    Article  PubMed  CAS  Google Scholar 

  7. Wigley FM, Wise RA, Seibold JR, McCloskey DA, Kujala G, Medsger Jr TA, Steen VD, Varga J, Jimenez S, Mayes M, Clements PJ, Weiner SR, Porter J, Ellman M, Wise C, Kaufman LD, Williams J, Dole W. Intravenous iloprost infusion in patients with Raynaud phenomenon secondary to systemic sclerosis. A multicenter, placebo-controlled, double-blind study. Ann Intern Med. 1994;120(3):199–206. PMID: 7506013.

    PubMed  CAS  Google Scholar 

  8. Steen VD, Medsger Jr TA. Case-control study of corticosteroids and other drugs that either precipitate or protect from the development of scleroderma renal crisis. Arthritis Rheum. 1998;41(9):1613–9. PMID: 9751093.

    Article  PubMed  CAS  Google Scholar 

  9. Steen VD, Mayes MD, Merkel PA. Assessment of kidney involvement. Clin Exp Rheumatol. 2003;21(3 Suppl 29):S29–31. PMID: 12889219.

    PubMed  CAS  Google Scholar 

  10. DeMarco PJ, Weisman MH, Seibold JR, Furst DE, Wong WK, Hurwitz EL, Mayes M, White B, Wigley F, Barr W, Moreland L, Medsger Jr TA, Steen V, Martin RW, Collier D, Weinstein A, Lally E, Varga J, Weiner SR, Andrews B, Abeles M, Clements PJ. Predictors and outcomes of scleroderma renal crisis: the high-dose versus low-dose D-penicillamine in early diffuse systemic sclerosis trial. Arthritis Rheum. 2002;46(11):2983–9. PMID: 12428241.

    Article  PubMed  CAS  Google Scholar 

  11. Mukerjee D, St George D, Coleiro B, Knight C, Denton CP, Davar J, Black CM, Coghlan JG. Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis. 2003;62(11):1088–93. PMID: 14583573.

    Article  PubMed  CAS  Google Scholar 

  12. Hachulla E, Launay D, Mouthon L, Sitbon O, Berezne A, Guillevin L, Hatron PY, Simonneau G, Clerson P, Humbert M. French PAH-scleroderma network. Is pulmonary arterial hypertension really a late complication of systemic sclerosis? Chest. 2009;136(5):1211–9, PMID: 19429720.

    Article  PubMed  Google Scholar 

  13. Williams MH, Das C, Handler CE, Akram MR, Davar J, Denton CP, Smith CJ, Black CM, Coghlan JG. Systemic sclerosis associated pulmonary hypertension: improved survival in the current era. Heart. 2006;92(7):926–32. PMID: 16339813.

    Article  PubMed  CAS  Google Scholar 

  14. Poole JL. Musculoskeletal rehabilitation in the person with scleroderma. Curr Opin Rheumatol. 2010;22(2):205–12. Review.PMID: 20019617.

    Article  PubMed  Google Scholar 

  15. Tyndall AJ, Bannert B, Vonk M, Airò P, Cozzi F, Carreira PE, Bancel DF, Allanore Y, Müller-Ladner U, Distler O, Iannone F, Pellerito R, Pileckyte M, Miniati I, Ananieva L, Gurman AB, Damjanov N, Mueller A, Valentini G, Riemekasten G, Tikly M, Hummers L, Henriques MJ, Caramaschi P, Scheja A, Rozman B, Ton E, Kumánovics G, Coleiro B, Feierl E, Szucs G, Von Mühlen CA, Riccieri V, Novak S, Chizzolini C, Kotulska A, Denton C, Coelho PC, Kötter I, Simsek I, de la Pena Lefebvre PG, Hachulla E, Seibold JR, Rednic S, Stork J, Morovic-Vergles J, Walker UA. Causes and risk factors for death in systemic sclerosis: a study from the EULAR scleroderma trials and research (EUSTAR) database. Ann Rheum Dis. 2010;69(10):1809–15, PMID: 20551155.

    Article  PubMed  Google Scholar 

  16. Domsic R, Fasanella K, Bielefeldt K. Gastrointestinal manifestations of systemic sclerosis. Dig Dis Sci. 2008;53(5):1163–74. Review.PMID: 17934861.

    Article  PubMed  Google Scholar 

  17. Ingraham KM, O’Brien MS, Shenin M, Derk CT, Steen VD. Gastric antral vascular ectasia in systemic sclerosis: demographics and disease predictors. J Rheumatol. 2010;37(3):603–7, PMID: 20080908.

    Article  PubMed  Google Scholar 

  18. Marie I, Ducrotte P, Antonietti M, Herve S, Levesque H. Watermelon stomach in systemic sclerosis: its incidence and management. Aliment Pharmacol Ther. 2008;28(4):412–21, PMID: 18498445.

    Article  PubMed  CAS  Google Scholar 

  19. Assassi S, Fritzler MJ, Arnett FC, Norman GL, Shah KR, Gourh P, Manek N, Perry M, Ganesh D, Rahbar MH, Mayes MD. Primary biliary cirrhosis (PBC), PBC autoantibodies, and hepatic parameter abnormalities in a large population of systemic sclerosis patients. J Rheumatol. 2009;36(10):2250–6, PMID: 19723904.

    Article  PubMed  Google Scholar 

  20. Ranque B, Authier FJ, Berezne A, Guillevin L, Mouthon L. Systemic sclerosis-associated myopathy. Ann N Y Acad Sci. 2007;1108:268–82. Review.PMID: 17899625.

    Article  PubMed  CAS  Google Scholar 

  21. Thombs BD, van Lankveld W, Bassel M, Baron M, Buzza R, Haslam S, Haythornthwaite JA, Hudson M, Jewett LR, Knafo R, Kwakkenbos L, Malcarne VL, Milette K, Motivala SJ, Newton EG, Nielson WR, Pacy M, Razykov I, Schieir O, Taillefer S, Worron-Sauve M. Psychological health and well-being in systemic sclerosis: state of the science and consensus research agenda. Arthritis Care Res (Hoboken). 2010;62(8):1181–9, PMID: 20235217.

    Article  Google Scholar 

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Correspondence to Maureen D. Mayes MD, MPH .

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© 2012 Springer Science+Business Media, LLC

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Mayes, M.D. (2012). Principles of Management. In: Varga, J., Denton, C., Wigley, F. (eds) Scleroderma. Springer, Boston, MA. https://doi.org/10.1007/978-1-4419-5774-0_47

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  • DOI: https://doi.org/10.1007/978-1-4419-5774-0_47

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  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4419-5773-3

  • Online ISBN: 978-1-4419-5774-0

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