From a clinical point of view, scleroderma is usually divided into two main forms, localized scleroderma and systemic scleroderma or systemic sclerosis (Figure 17A-1). Localized scleroderma includes the disease entities of morphea (one or more patches of thickened skin), linear scleroderma (a line of thickened skin affecting one or more extremities), and scleroderma en coup de sabre, which is a distinct subset of linear disease that affects the forehead and face [for review, see Piette (1)]. Although atrophy of the subcutaneous tissue underlying the lesions typically occurs in localized scleroderma, there is usually no associated internal organ or systemic involvement.


Pulmonary Arterial Hypertension Interstitial Lung Disease Systemic Sclerosis Scleroderma Renal Crisis Localize Scleroderma 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. 1.
    Piette WW. Morphea or localized scleroderma. In: Clements PJ, Furst DE, eds. Systemic sclerosis. Philadelphia: Lippincott Williams & Wilkins; 2004:29–37.Google Scholar
  2. 2.
    Medsger TA Jr. Classification, prognosis. In: Clements PJ, Furst DE, eds. Systemic sclerosis. Philadelphia: Lippincott Williams & Wilkins; 2004:17–28.Google Scholar
  3. 3.
    Korn JH. Pathogenesis of systemic sclerosis. In: Koopman WJ, Moreland LW, eds. Arthritis and allied conditions. Philadelphia: Lippincott Williams & Wilkins; 2005:1621–1632.Google Scholar
  4. 4.
    Boin F, Wigley FM. Understanding, assessing and treating Raynaud’s phenomenon. Curr Opin Rheumatol 2005;17:752–760.PubMedGoogle Scholar
  5. 5.
    Fraenkel L, Zhang Y, Chaisson CE, et al. Different factors influencing the expression of Raynaud’s phenomenon in men and women. Arthritis Rheum 1999;42:306–310.CrossRefPubMedGoogle Scholar
  6. 6.
    Suter LG, Murabito JM, Felson DT, Fraenkel L. The incidence and natural history of Raynaud’s phenomenon in the community. Arthritis Rheum 2005;52:1259–1263.CrossRefPubMedGoogle Scholar
  7. 7.
    Subcommittee for Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee: preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 1980;23:581–590.Google Scholar
  8. 8.
    LeRoy EC, Black C, Fleischmajer R, et al. Scleroderma (systemic sclerosis): classification, subsets, and pathogenesis. J Rheumatol 1988;15:202–205.PubMedGoogle Scholar
  9. 9.
    Steen VD, Medsger TA Jr. Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum 2000;43:2437–2444.CrossRefPubMedGoogle Scholar
  10. 10.
    Bryan C, Knight C, Black CM, Silman AJ. Prediction of five-year survival following presentation with scleroderma: development of a simplet model using three disease factors at first visit. Arthritis Rheum 1999;42:2660–2665.CrossRefPubMedGoogle Scholar
  11. 11.
    Cepeda EJ, Reveille JD. Autoantibodies in systemic sclerosis and fibrosing syndromes: clinical indications and relevance. Curr Opin Rheumatol 2004;16:723–732.CrossRefPubMedGoogle Scholar
  12. 12.
    Clements PJ, Medsger TA Jr, Feghali CA. Cutaneous involvement in systemic sclerosis. In: Clements PJ, Furst DE, eds. Systemic sclerosis. Philadelphia: Lippincott Williams & Wilkins; 2004:129–150.Google Scholar
  13. 13.
    Poormoghim H, Lucas M, Fertig N, Medsger TA Jr. Systemic sclerosis sine scleroderma: demographic, clinical, and serologic features and survival in forty-eight patients. Arthritis Rheum 2000;43:444–451.CrossRefPubMedGoogle Scholar
  14. 14.
    Weinstein WM, Kadell BM. The gastrointestinal tract in systemic sclerosis. In: Clements PJ, Furst DE, eds. Systemic sclerosis. Philadelphia: Lippincott Williams & Wilkins; 2004:293–308.Google Scholar
  15. 15.
    Goldblatt F, Gordon TP, Waterman SA. Antibody-mediated gastrointestinal dysmotility in scleroderma. Gastroenterology 2002;123:1144–1150.CrossRefPubMedGoogle Scholar
  16. 16.
    Mackey RI. Autoimmunity and primary biliary cirrhosis. Baillieres Best Pract Res Clin Gastroenterol 2000;14:519–533.CrossRefGoogle Scholar
  17. 17.
    Chang B, Wigley FM, White B, Wise RA. Scleroderma patients with combined pulmonary hypertension and interstitial lung disease. J Rheumatol 2003;30:2398–2405.PubMedGoogle Scholar
  18. 18.
    Hachulla E, Gressin V, Guillevin L, et al. Early detection of pulmonary arterial hypertension in systemic sclero-sis: a French nationwide prospective multicenter study. Arthritis Rheum 2005;52:3792–3800.CrossRefPubMedGoogle Scholar
  19. 19.
    Mukerjee D, St George D, Coleiro B, et al. Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis 2003;62:1088–1093.CrossRefPubMedGoogle Scholar
  20. 20.
    Wigley FM, Lima JAC, Mayes M, McLain D, Chapin JL, Ward-Able C. The UNCOVER study: prevalence of undiagnosed pulmonary arterial hypertension in subjects with connective tissue disease at the secondary health-care level of community-based rheumatologists. Arthritis Rheum 2005;52:2125–2132.CrossRefPubMedGoogle Scholar
  21. 21.
    Chang B, Wigley FM, White B, Wise RA. Scleroderma patients with combined pulmonary hypertension and interstitial lung disease. J Rheumatol 2006;33:269–274.PubMedGoogle Scholar
  22. 22.
    Follansbee WP, Marroquin OC. Cardiac involvement in systemic sclerosis. In: Clements PJ, Furst DE, eds. Systemic sclerosis. Philadelphia: Lippincott Williams & Wilkins; 2004:195–220.Google Scholar
  23. 23.
    Smith JW, Clements PJ, Levisman J, Furst D, Ross M. Echocardiographic features of progressive systemic sclerosis (PSS): correlation with hemodynamic and postmortem studies. Am J Med 1979;66:28–33.CrossRefPubMedGoogle Scholar
  24. 24.
    Steen VD. Renal involvement in systemic sclerosis. In: Clements PJ, Furst DE, eds. Systemic sclerosis. Philadelphia: Lippincott Williams & Wilkins; 2004:279–292.Google Scholar
  25. 25.
    Pope JE. Musculoskeletal involvement in scleroderma. Rheum Dis Clin North Am 2003;52:391–405.CrossRefGoogle Scholar
  26. 26.
    Clements PJ, Furst DE, Campion DS, et al. Muscle disease in progressive systemic sclerosis: diagnostic and therapeutic consideration. Arthritis Rheum 1978;21:62–71.CrossRefPubMedGoogle Scholar
  27. 27.
    Mori Y, Kahari VM, Varga J. Scleroderma-like cutaneous syndromes. Curr Rheumatol Rep 2002;4:113–122.CrossRefPubMedGoogle Scholar
  28. 28.
    Cowper SE, Boyer PJ. Nephrogenic systemic fibrosis: an update. Curr Rheumatol Rep 2006;8:151–157.CrossRefPubMedGoogle Scholar
  29. 1.
    Maricq HR, Weinrich MC, Keil JE, et al. Prevalence of scleroderma spectrum disorders in the general population of South Carolina. Arthritis Rheum 1989;32:998–1006.CrossRefPubMedGoogle Scholar
  30. 2.
    Mayes MD, Lacey JV Jr, Beebe-Dimmer J, et al. Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum 2003;48:2246–2255.CrossRefPubMedGoogle Scholar
  31. 3.
    Kuwana M, Kaburaki J, Arnett FC, Howard RF, Medsger TA Jr, Wright TM. Influence of ethnic background on clinical and serologic features in patients with systemic sclerosis and anti-DNA topoisomerase I antibody. Arthritis Rheum 1999;42:465–474.CrossRefPubMedGoogle Scholar
  32. 4.
    Feghali-Bostwick C, Medsger TA Jr, Wright TM. Analysis of systemic sclerosis in twins reveals low concordance for disease and high concordance for the presence of anti-nuclear antibodies. Arthritis Rheum 2003;48:1956–1963.CrossRefPubMedGoogle Scholar
  33. 5.
    Janowsky EC, Kupper LL, Hulka BS. Meta-analyses of the relation between silicone breast implants and the risk of connective-tissue diseases. N Engl J Med 2000;342:781–790.CrossRefPubMedGoogle Scholar
  34. 6.
    Prescott RJ, Freemont AJ, Jones CJ, Hoyland J, Fielding P. Sequential dermal microvascular and perivascular changes in the development of scleroderma. J Pathol 1992;166:255–263.CrossRefPubMedGoogle Scholar
  35. 7.
    Abraham DJ, Varga J. Scleroderma: from cell and molecular mechanisms to disease models. Trends Immunol 2005;26:587–595.CrossRefPubMedGoogle Scholar
  36. 8.
    Kuwana M, Okazaki Y, Yasuoka H, Kawakami Y, Ikeda Y. Defective vasculogenesis in systemic sclerosis. Lancet 2004;364:603–610.CrossRefPubMedGoogle Scholar
  37. 9.
    Sato S, Fujimoto M, Hasegawa M, Takehara K. Altered blood B lymphocyte homeostasis in systemic sclerosis: expanded naive B cells and diminished but activated memory B cells. Arthritis Rheum 2004;50:1918–1927.CrossRefPubMedGoogle Scholar
  38. 10.
    Abe R, Donnelly SC, Peng T, Bucala R, Metz CN. Peripheral blood fibrocytes: differentiation pathway and migration to wound sites. J Immunol 2001;166:7556–7562.PubMedGoogle Scholar
  39. 11.
    Whitfield ML, Finlay DR, Murray JI, et al. Systemic and cell type-specific gene expression patterns in SSc skin. Proc Natl Acad Sci U S A. 2003;100:12319–12324.CrossRefPubMedGoogle Scholar
  40. 12.
    Bhattacharyya S, Ghosh AK, Pannu J, et al. Fibroblast expression of the coactivator p300 governs the intensity of profibrotic response to transforming growth factor beta. Arthritis Rheum 2005;52:1248–1258.CrossRefPubMedGoogle Scholar
  41. 1.
    McGoon M, Gutterman D, Steen V, et al. Screening, early detection, and diagnosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004;126:11S–34S.CrossRefGoogle Scholar
  42. 2.
    Farber HW, Loscalzo J. Pulmonary arterial hypertension. N Engl J Med 2004;351:1655–1665.CrossRefPubMedGoogle Scholar
  43. 3.
    DeMarco PJ, Weisman M, Seibold JR, et al. Predictors and outcome of scleroderma renal crisis. The high-dose versus low-dose D-penicillamine in early diffuse systemic sclerosis trial. Arthritis Rheum 2002;46:2983–2989.CrossRefPubMedGoogle Scholar
  44. 4.
    Korn JH, Mayes M, Matucci-Cerinic M, et al. Digital ulcers in systemic sclerosis—prevention by treatment with bosentan, an oral endothelin receptor antagonist. Arthritis Rheum 2004;50:3985–3993.CrossRefPubMedGoogle Scholar
  45. 5.
    Tashkin DP, Elashoff R, Clements PJ, et al. The Scleroderma Lung Study: oral cyclophosphamide versus placebo for the treatment of scleroderma-related interstitial lung disease. N Engl J Med 2006;354:2655–2666.CrossRefPubMedGoogle Scholar
  46. 6.
    McSweeney PA, Nash RA, Sullivan KM, et al. High-dose immunosuppressive therapy for severe systemic sclerosis: initial outcomes. Blood 2002;100:1602–1610.PubMedGoogle Scholar
  47. 7.
    Pope JE, Bellamy N, Seibold JR, et al. A randomized controlled trial of methotrexate versus placebo in early diffuse scleroderma. Arthritis Rheum 2001;44:1351–1358.CrossRefPubMedGoogle Scholar
  48. 8.
    Stratton RJ, Wilson H, Black CM. Pilot study of anti-thymocyte globulin plus mycophenolate mofetil in recent-onset diffuse scleroderma. Rheumatology (Oxford) 2001;40:84–88.CrossRefGoogle Scholar
  49. 9.
    Clements PJ, Furst DE, Wong W-K, et al. High-dose versus low-dose D-penicillamine in early diffuse systemic sclerosis. Arthritis Rheum 1999;42:1194–1203.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2008

Authors and Affiliations

  • Maureen D. Mayes
    • 1
  1. 1.Department Of Internal Medicine, Division Of Rheumatology And ImmunogeneticsUniversity Of Texas-Houston Medical SchoolHoustonUSA

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