Scleroderma 

  • Author: Sergio A Jimenez, MD, MACR; Chief Editor: Herbert S Diamond, MD   more...
 
Updated: Jul 22, 2011
 

Background

Scleroderma is derived from the Greek words skleros (hard or indurated) and derma (skin). Hippocrates first described this condition as thickened skin.[1] Carlo Curzio (1752) offered the first detailed description of scleroderma in a patient with hard skin, which he described as woodlike or as containing a dry hide. In 1836, Giovambattista Fantonetti applied the term scleroderma to a patient's condition.[2] He applied the term to describe a patient with dark leatherlike skin who exhibited a loss of range of joint motion due to skin tightening. Robert H. Goetz first described in detail the concept of scleroderma as a systemic disease in 1945; he introduced the term progressive systemic sclerosis to emphasize the systemic and often progressive nature of the disease.

Definition

The term systemic sclerosis is used to describe a systemic disease characterized by skin induration and thickening accompanied by various degrees of tissue fibrosis and chronic inflammatory infiltration in numerous visceral organs, prominent fibroproliferative vasculopathy, and humoral and cellular immune alterations.

The American College of Rheumatology (ACR) criteria for the classification of systemic sclerosis require one major criterion or two minor criteria, as follows:

  • Major criterion: Proximal scleroderma is characterized by symmetric thickening, tightening, and induration of the skin of the fingers and the skin that is proximal to the metacarpophalangeal or metatarsophalangeal joints. These changes may affect the entire extremity, face, neck, and trunk (thorax and abdomen; see images below). Tightening of the skin in the face, with a charactTightening of the skin in the face, with a characteristic beaklike facies and paucity of wrinkles. Sclerodactyly with digital ulceration, loss of skiSclerodactyly with digital ulceration, loss of skin creases, joint contractures, and sparse hair.
  • Minor criteria
    • Sclerodactyly is characterized by thickening, induration, and tightening of the skin, limited to only the fingers.
    • Digital pitting scars or a loss of substance from the finger pad: As a result of ischemia, depressed areas of the fingertips or a loss of digital pad tissue occurs.
    • Bibasilar pulmonary fibrosis includes a bilateral reticular pattern of linear or lineonodular densities most pronounced in basilar portions of the lungs on standard chest roentgenography. These densities may assume the appearance of diffuse mottling or a honeycomb lung and are not attributable to primary lung disease.
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Pathophysiology

Systemic sclerosis is a systemic disease that affects many organ systems. It is most obvious in the skin; however, the GI tract; the respiratory, renal, cardiovascular, and genitourinary systems; and numerous vascular structures are frequently involved. The symptoms result from inflammation and progressive tissue fibrosis and occlusion of the microvasculature by excessive production and deposition of types I and III collagens. The levels of other macromolecules (eg, glycosaminoglycans, tenascin, fibronectin) found in the connective tissue are also increased.

The vascular alterations show a predilection for the small arteries and arterioles. Vascular dysfunction is one of the earliest alterations of systemic sclerosis and may represent the initiating event in its pathogenesis. Severe alterations in small blood vessels of skin and internal organs, including fibrosis and perivascular cellular infiltration with activated T cells, are almost always present in systemic sclerosis.

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Epidemiology

Frequency

United States

The estimated incidence of systemic sclerosis is 19 cases per million population, and the prevalence of systemic sclerosis has been estimated at 240 cases per million population, although the reported prevalence has ranged from 138 to 286 cases per million population. The prevalence has increased because of earlier detection through better diagnosis and an increased survival rate. Obtaining an exact estimate of prevalence is difficult because systemic sclerosis is frequently misdiagnosed. Juvenile-onset systemic sclerosis is uncommon and usually presents as an overlap syndrome with myositis. In the United States, the prevalence of systemic sclerosis has been reported to be as high as 400 cases per million women aged 35-65 years.

International

Systemic sclerosis is found worldwide.

Mortality/Morbidity

Pulmonary hypertension, pulmonary fibrosis (interstitial lung disease), and scleroderma renal crisis are the most frequent causes of mortality. Survival averages 12 years from diagnosis and correlates best with the clinical disease subtype (diffuse cutaneous vs limited cutaneous) and extent of organ involvement.

  • The limited cutaneous subset carries a 10-year survival rate of 71%.
  • The diffuse cutaneous subset carries a 10-year survival rate of 21%.
  • Pulmonary hypertension is a major prognostic factor for survival.

Race

Systemic sclerosis affects individuals of all races.

  • The risk of systemic sclerosis in blacks is slightly higher than in whites; in young black women, the risk is 10 times higher.
  • Incidence rates among ethnically related groups who are geographically separate show some discrepancy. The incidence of systemic sclerosis is lower in native Nigerians than in African Americans. Oklahoma Choctaw Indians have an incidence of 472 cases per million population, which is higher than that of the Missouri Choctaw Indians. These differences may be due to environmental exposures or differences in genetic predisposition.

Sex

The risk of systemic sclerosis is 4-9 times higher in women than in men.

Age

The peak onset occurs in individuals aged 30-50 years.

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Contributor Information and Disclosures
Author

Sergio A Jimenez, MD, MACR  Fellow, Royal College of Physicians United Kingdom, Director, The Scleroderma Center; Professor and Co-Director, Jefferson Institute of Molecular Medicine; Professor of Biochemistry and Molecular Biology; Director, Division of Connective Tissue Diseases, Department of Dermatology and Cutaneous Biology, Thomas Jefferson University

Sergio A Jimenez, MD, MACR is a member of the following medical societies: American College of Physicians, American College of Rheumatology, American Society for Biochemistry and Molecular Biology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

Patrick M Cronin, DO, FACR  Clinical Associate Professor of Medicine, Department of Medicine, Division of Rheumatology, University of Pennsylvania Health System, Pennsylvania Hospital

Patrick M Cronin, DO, FACR is a member of the following medical societies: American College of Rheumatology, American Osteopathic Association, and Pennsylvania Medical Society

Disclosure: Nothing to disclose.

Andrew S Koenig, DO  Consulting Staff, Division of Rheumatology, Rancoccas Hospital

Andrew S Koenig, DO is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine, American College of Rheumatology, and American Osteopathic Association

Disclosure: Nothing to disclose.

Marie Spevak O'Brien, DO  Assistant Clinical Professor of Medicine, Arthritis and Rheumatology, Lehigh Valley Physician Group

Marie Spevak O'Brien, DO is a member of the following medical societies: American College of Physicians, American College of Rheumatology, American Medical Association, American Osteopathic Association, International Society for Clinical Densitometry, and Pennsylvania Medical Society

Disclosure: Nothing to disclose.

Specialty Editor Board

John Varga, MD  Professor, Department of Internal Medicine, Division of Rheumatology, Northwestern University

John Varga, MD is a member of the following medical societies: American College of Physicians, American College of Rheumatology, Central Society for Clinical Research, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Elliot Goldberg, MD  Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine, Temple University School of Medicine

Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, and American College of Rheumatology

Disclosure: Nothing to disclose.

Alex J Mechaber, MD, FACP  Senior Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD  Adjunct Professor of Medicine, Division of Rheumatology, University of Pittsburgh School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa

Disclosure: Merck Ownership interest Other; Smith Kline Ownership interest Other; Zimmer Ownership interest Other

Acknowledgments

The expert assistance of Susan V. Castro, PhD, in preparation of the manuscript is gratefully acknowledged.

Sergio A Jimenez, MD, was recipient of NIH/NIAMS grant #RO-1 AR19616.

References

  1. David M. A case of scleroderma mentioned by Hippocrates in his aphorisms. Korot. 1981;8(1-2):61-3. [Medline].

  2. Fantonetti G. Case of general induration of the skin. Dublin J Med Sci. 1838;13:158-9.

  3. Pope JE. Musculoskeletal involvement in scleroderma. Rheum Dis Clin North Am. May 2003;29(2):391-408. [Medline].

  4. Derk CT, Rasheed M, Spiegel JR, et al. Increased incidence of carcinoma of the tongue in patients with systemic sclerosis. J Rheumatol. Apr 2005;32(4):637-41. [Medline].

  5. Derk CT, Rasheed M, Artlett CM, et al. A cohort study of cancer incidence in systemic sclerosis. J Rheumatol. Jun 2006;33(6):1113-6. [Medline].

  6. Wooten M. Systemic sclerosis and malignancy: a review of the literature. South Med J. Jan 2008;101(1):59-62. [Medline].

  7. Au K, Singh MK, Bodukam V, et al. Atherosclerosis in systemic sclerosis: A systematic review and meta-analysis. Arthritis Rheum. Jul 2011;63(7):2078-90. [Medline]. [Full Text].

  8. Hsu VM, Moreyra AE, Wilson AC, et al. Assessment of pulmonary arterial hypertension in patients with systemic sclerosis: comparison of noninvasive tests with results of right-heart catheterization. J Rheumatol. Mar 2008;35(3):458-65. [Medline].

  9. Launay D, Remy-Jardin M, Michon-Pasturel U, et al. High resolution computed tomography in fibrosing alveolitis associated with systemic sclerosis. J Rheumatol. Sep 2006;33(9):1789-801. [Medline].

  10. Rigamonti C, Shand LM, Feudjo M, et al. Clinical features and prognosis of primary biliary cirrhosis associated with systemic sclerosis. Gut. Mar 2006;55(3):388-94. [Medline].

  11. Nietert PJ, Sutherland SE, Silver RM, et al. Is occupational organic solvent exposure a risk factor for scleroderma?. Arthritis Rheum. Jun 1998;41(6):1111-8. [Medline].

  12. Perera A, Fertig N, Lucas M, et al. Clinical subsets, skin thickness progression rate, and serum antibody levels in systemic sclerosis patients with anti-topoisomerase I antibody. Arthritis Rheum. Aug 2007;56(8):2740-6. [Medline].

  13. Arnett FC. Is scleroderma an autoantibody mediated disease?. Curr Opin Rheumatol. Nov 2006;18(6):579-81. [Medline].

  14. Baroni SS, Santillo M, Bevilacqua F, et al. Stimulatory autoantibodies to the PDGF receptor in systemic sclerosis. N Engl J Med. Jun 22 2006;354(25):2667-76. [Medline].

  15. Allanore Y, Borderie D, Meune C, et al. N-terminal pro-brain natriuretic peptide as a diagnostic marker of early pulmonary artery hypertension in patients with systemic sclerosis and effects of calcium-channel blockers. Arthritis Rheum. Dec 2003;48(12):3503-8. [Medline].

  16. Strange C, Bolster MB, Roth MD, et al. Bronchoalveolar lavage and response to cyclophosphamide in scleroderma interstitial lung disease. Am J Respir Crit Care Med. Jan 1 2008;177(1):91-8. [Medline].

  17. Nash RA, McSweeney PA, Nelson JL, et al. Allogeneic marrow transplantation in patients with severe systemic sclerosis: resolution of dermal fibrosis. Arthritis Rheum. Jun 2006;54(6):1982-6. [Medline].

  18. [Best Evidence] Fries R, Shariat K, von Wilmowsky et al. Sildenafil in the treatment of Raynaud's phenomenon resistant to vasodilatory therapy. Circulation. Nov 8 2005;112(19):2980-5. [Medline].

  19. Badesch DB, Hill NS, Burgess G, et al. Sildenafil for pulmonary arterial hypertension associated with connective tissue disease. J Rheumatol. Dec 2007;34(12):2417-22. [Medline].

  20. Tashkin DP, Elashoff R, Clements PJ, et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med. Jun 22 2006;354(25):2655-66. [Medline].

  21. Liossis SN, Bounas A, Andonopoulos AP. Mycophenolate mofetil as first-line treatment improves clinically evident early scleroderma lung disease. Rheumatology (Oxford). Aug 2006;45(8):1005-8. [Medline].

  22. Oldfield V, Lyseng-Williamson KA. Bosentan: a review of its use in pulmonary arterial hypertension and systemic sclerosis. Am J Cardiovasc Drugs. 2006;6(3):189-208. [Medline].

  23. Swigris JJ, Olson AL, Fischer A, et al. Mycophenolate mofetil is safe, well tolerated, and preserves lung function in patients with connective tissue disease-related interstitial lung disease. Chest. Jul 2006;130(1):30-6. [Medline].

  24. Gerbino AJ, Goss CH, Molitor JA. Effect of mycophenolate mofetil on pulmonary function in scleroderma-associated interstitial lung disease. Chest. Feb 2008;133(2):455-60. [Medline].

  25. Sitbon O, Badesch DB, Channick RN, et al. Effects of the dual endothelin receptor antagonist bosentan in patients with pulmonary arterial hypertension: a 1-year follow-up study. Chest. Jul 2003;124(1):247-54. [Medline].

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

  27. Schachna L, Medsger TA Jr, Dauber JH, et al. Lung transplantation in scleroderma compared with idiopathic pulmonary fibrosis and idiopathic pulmonary arterial hypertension. Arthritis Rheum. Dec 2006;54(12):3954-61. [Medline].

  28. Vonk MC, Marjanovic Z, van den Hoogen FH, et al. Long-term follow-up results after autologous haematopoietic stem cell transplantation for severe systemic sclerosis. Ann Rheum Dis. Jan 2008;67(1):98-104. [Medline].

  29. Passweg J, Tyndall A. Autologous stem cell transplantation in autoimmune diseases. Semin Hematol. Oct 2007;44(4):278-85. [Medline].

  30. Kissin EY, Schiller AM, Gelbard RB, et al. Durometry for the assessment of skin disease in systemic sclerosis. Arthritis Rheum. Aug 15 2006;55(4):603-9. [Medline].

  31. American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Arthritis Rheum. May 1980;23(5):581-90. [Medline].

  32. Barnett AJ, Miller MH, Littlejohn GO. A survival study of patients with scleroderma diagnosed over 30 years (1953-1983): the value of a simple cutaneous classification in the early stages of the disease. J Rheumatol. Feb 1988;15(2):276-83. [Medline].

  33. Bogoch ER, Gross DK. Surgery of the hand in patients with systemic sclerosis: outcomes and considerations. J Rheumatol. Apr 2005;32(4):642-8. [Medline].

  34. Boin F, Hummers LK. Scleroderma-like fibrosing disorders. Rheum Dis Clin North Am. Feb 2008;34(1):199-220; ix. [Medline].

  35. Cepeda EJ, Reveille JD. Autoantibodies in systemic sclerosis and fibrosing syndromes: clinical indications and relevance. Curr Opin Rheumatol. Nov 2004;16(6):723-32. [Medline].

  36. Champion HC. The heart in scleroderma. Rheum Dis Clin North Am. Feb 2008;34(1):181-90; viii. [Medline].

  37. Chifflot H, Fautrel B, Sordet C, et al. Incidence and prevalence of systemic sclerosis: a systematic literature review. Semin Arthritis Rheum. Feb 2008;37(4):223-35. [Medline].

  38. Czirjak L, Kumanovics G, Varju C, et al. Survival and causes of death in 366 Hungarian patients with systemic sclerosis. Ann Rheum Dis. Jan 2008;67(1):59-63. [Medline].

  39. Denton CP, Black CM. Pulmonary hypertension in systemic sclerosis. Rheum Dis Clin North Am. May 2003;29(2):335-49, vii. [Medline].

  40. Derk CT, Huaman G, Jimenez SA. A retrospective randomly selected cohort study of D-penicillamine treatment in rapidly progressive diffuse cutaneous systemic sclerosis of recent onset. Br J Dermatol. May 2008;158(5):1063-8. [Medline].

  41. Derk CT, Jimenez SA. Systemic sclerosis: current views of its pathogenesis. Autoimmun Rev. Jun 2003;2(4):181-91. [Medline].

  42. Deswal A, Follansbee WP. Cardiac involvement in scleroderma. Rheum Dis Clin North Am. Nov 1996;22(4):841-60. [Medline].

  43. Di Ciaula A, Covelli M, Berardino M, et al. Gastrointestinal symptoms and motility disorders in patients with systemic scleroderma. BMC Gastroenterol. Feb 27 2008;8:7. [Medline].

  44. Distler J, Distler O. Novel treatment approaches to fibrosis in scleroderma. Rheum Dis Clin North Am. Feb 2008;34(1):145-59; vii. [Medline].

  45. Distler JH, Hoeper MM, Distler O. Diagnosis of pulmonary arterial hypertension in a patient with systemic sclerosis. Nat Clin Pract Rheumatol. Mar 2008;4(3):160-4. [Medline].

  46. Distler O, Behrens F, Huscher D, et al. Need for improved outcome measures in pulmonary arterial hypertension related to systemic sclerosis. Rheumatology (Oxford). Dec 2006;45(12):1455-7. [Medline].

  47. Domsic R, Fasanella K, Bielefeldt K. Gastrointestinal manifestations of systemic sclerosis. Dig Dis Sci. May 2008;53(5):1163-74. [Medline].

  48. Ebert EC. Gastric and enteric involvement in progressive systemic sclerosis. J Clin Gastroenterol. Jan 2008;42(1):5-12. [Medline].

  49. Gerbracht DD, Steen VD, Ziegler GL, et al. Evolution of primary Raynaud's phenomenon (Raynaud's disease) to connective tissue disease. Arthritis Rheum. Jan 1985;28(1):87-92. [Medline].

  50. Goh NS, Desai SR, Veeraraghavan S, et al. Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med. Jun 1 2008;177(11):1248-54. [Medline].

  51. Guiducci S, Giacomelli R, Cerinic MM. Vascular complications of scleroderma. Autoimmun Rev. Sep 2007;6(8):520-3. [Medline].

  52. Hachulla E, Coghlan JG. A new era in the management of pulmonary arterial hypertension related to scleroderma: endothelin receptor antagonism. Ann Rheum Dis. Sep 2004;63(9):1009-14. [Medline].

  53. Herrick AL. Pathogenesis of Raynaud's phenomenon. Rheumatology (Oxford). May 2005;44(5):587-96. [Medline].

  54. Hettema ME, Bootsma H, Kallenberg CG. Macrovascular disease and atherosclerosis in SSc. Rheumatology (Oxford). May 2008;47(5):578-83. [Medline].

  55. Highland KB, Garin MC, Brown KK. The spectrum of scleroderma lung disease. Semin Respir Crit Care Med. Aug 2007;28(4):418-29. [Medline].

  56. Hoyles RK, Ellis RW, Wellsbury J, et al. A multicenter, prospective, randomized, double-blind, placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma. Arthritis Rheum. Dec 2006;54(12):3962-70. [Medline].

  57. Janiak P, Thumshirn M, Menne D, et al. Clinical trial: the effects of adding ranitidine at night to twice daily omeprazole therapy on nocturnal acid breakthrough and acid reflux in patients with systemic sclerosis--a randomized controlled, cross-over trial. Aliment Pharmacol Ther. Nov 1 2007;26(9):1259-65. [Medline].

  58. Jaovisidha K, Csuka ME, Almagro UA, et al. Severe gastrointestinal involvement in systemic sclerosis: report of five cases and review of the literature. Semin Arthritis Rheum. Feb 2005;34(4):689-702. [Medline].

  59. Jimenez SA, Derk CT. Following the molecular pathways toward an understanding of the pathogenesis of systemic sclerosis. Ann Intern Med. Jan 6 2004;140(1):37-50. [Medline].

  60. Johnson SR, Feldman BM, Hawker GA. Classification criteria for systemic sclerosis subsets. J Rheumatol. Sep 2007;34(9):1855-63. [Medline].

  61. Khurma V, Meyer C, Park GS, et al. A pilot study of subclinical coronary atherosclerosis in systemic sclerosis: coronary artery calcification in cases and controls. Arthritis Rheum. Apr 15 2008;59(4):591-7. [Medline].

  62. Knockaert DC. Cardiac involvement in systemic inflammatory diseases. Eur Heart J. Aug 2007;28(15):1797-804. [Medline].

  63. Lawrence RC, Helmick CG, Arnett FC, et al. Estimates of the prevalence of arthritis and selected musculoskeletal disorders in the United States. Arthritis Rheum. May 1998;41(5):778-99. [Medline].

  64. Manetti M, Neumann E, Milia AF, et al. Severe fibrosis and increased expression of fibrogenic cytokines in the gastric wall of systemic sclerosis patients. Arthritis Rheum. Oct 2007;56(10):3442-7. [Medline].

  65. Mayes MD. Scleroderma epidemiology. Rheum Dis Clin North Am. May 2003;29(2):239-54. [Medline].

  66. Medsger TA Jr. Natural history of systemic sclerosis and the assessment of disease activity, severity, functional status, and psychologic well-being. Rheum Dis Clin North Am. May 2003;29(2):255-73, vi. [Medline].

  67. Medsger TA Jr, Masi AT. Survival with scleroderma. II. A life-table analysis of clinical and demographic factors in 358 male U.S. veteran patients. J Chronic Dis. Oct 1973;26(10):647-60. [Medline].

  68. Nihtyanova SI, Denton CP. Current approaches to the management of early active diffuse scleroderma skin disease. Rheum Dis Clin North Am. Feb 2008;34(1):161-79; viii. [Medline].

  69. Parodi A, Sessarego M, Greco A, et al. Small intestinal bacterial overgrowth in patients suffering from scleroderma: clinical effectiveness of its eradication. Am J Gastroenterol. May 2008;103(5):1257-62. [Medline].

  70. Penn H, Howie AJ, Kingdon EJ, et al. Scleroderma renal crisis: patient characteristics and long-term outcomes. QJM. Aug 2007;100(8):485-94. [Medline].

  71. Poncelet AN, Connolly MK. Peripheral neuropathy in scleroderma. Muscle Nerve. Sep 2003;28(3):330-5. [Medline].

  72. Rodnan GP, Benedek TG. An historical account of the study of progressive systemic sclerosis (diffuse scleroderma). Ann Intern Med. Aug 1962;57:305-19. [Medline].

  73. Rose RF, Goodfield MJ. Combining PUVA therapy with systemic immunosuppression to treat progressive diffuse morphoea. Clin Exp Dermatol. May 2005;30(3):226-8. [Medline].

  74. Rubin LJ. Treatment of pulmonary arterial hypertension due to scleroderma: challenges for the future. Rheum Dis Clin North Am. Feb 2008;34(1):191-7; viii. [Medline].

  75. Scalapino K, Arkachaisri T, Lucas M, et al. Childhood onset systemic sclerosis: classification, clinical and serologic features, and survival in comparison with adult onset disease. J Rheumatol. May 2006;33(5):1004-13. [Medline].

  76. Schwarzer AC, Derby R, Aprill CN, et al. The value of the provocation response in lumbar zygapophyseal joint injections. Clin J Pain. Dec 1994;10(4):309-13. [Medline].

  77. Shah AA, Wigley FM. Often forgotten manifestations of systemic sclerosis. Rheum Dis Clin North Am. Feb 2008;34(1):221-38; ix. [Medline].

  78. Steen V. Predictors of end stage lung disease in systemic sclerosis. Ann Rheum Dis. Feb 2003;62(2):97-9. [Medline].

  79. Steen VD. Scleroderma and pregnancy. Rheum Dis Clin North Am. Feb 1997;23(1):133-47. [Medline].

  80. Steen VD. Scleroderma renal crisis. Rheum Dis Clin North Am. May 2003;29(2):315-33. [Medline].

  81. Steen VD, Brodeur M, Conte C. Prospective pregnancy (PG) study in women with systemic sclerosis (SSc). Arthr Rheum. 1996;39:5151.

  82. Steen VD, Syzd A, Johnson JP, et al. Kidney disease other than renal crisis in patients with diffuse scleroderma. J Rheumatol. Apr 2005;32(4):649-55. [Medline].

  83. Strange C, Highland KB. Interstitial lung disease in the patient who has connective tissue disease. Clin Chest Med. Sep 2004;25(3):549-59, vii. [Medline].

  84. Thombs BD, Hudson M, Taillefer SS, et al. Prevalence and clinical correlates of symptoms of depression in patients with systemic sclerosis. Arthritis Rheum. Apr 15 2008;59(4):504-9. [Medline].

  85. [Best Evidence] Trad S, Amoura Z, Beigelman C, et al. Pulmonary arterial hypertension is a major mortality factor in diffuse systemic sclerosis, independent of interstitial lung disease. Arthritis Rheum. Jan 2006;54(1):184-91. [Medline].

  86. Tuchinda C, Kerr HA, Taylor CR, et al. UVA1 phototherapy for cutaneous diseases: an experience of 92 cases in the United States. Photodermatol Photoimmunol Photomed. Oct 2006;22(5):247-53. [Medline].

  87. van Wijk RM, Geurts JW, Wynne HJ, et al. Radiofrequency denervation of lumbar facet joints in the treatment of chronic low back pain: a randomized, double-blind, sham lesion-controlled trial. Clin J Pain. Jul-Aug 2005;21(4):335-44. [Medline].

  88. Varga JA, Trojanowska M. Fibrosis in systemic sclerosis. Rheum Dis Clin North Am. Feb 2008;34(1):115-43; vii. [Medline].

  89. Walker JG, Fritzler MJ. Update on autoantibodies in systemic sclerosis. Curr Opin Rheumatol. Nov 2007;19(6):580-91. [Medline].

  90. White B. Interstitial lung disease in scleroderma. Rheum Dis Clin North Am. May 2003;29(2):371-90. [Medline].

  91. Zulian F. Systemic sclerosis and localized scleroderma in childhood. Rheum Dis Clin North Am. Feb 2008;34(1):239-55; ix. [Medline].

 
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Tightening of the skin in the face, with a characteristic beaklike facies and paucity of wrinkles.
Sclerodactyly with digital ulceration, loss of skin creases, joint contractures, and sparse hair.
Anterior chest demonstrating salt-and-pepper hypopigmentation and diffuse hyperpigmentation in a white woman.
A radiograph of the distal digits demonstrating calcinosis and distal phalanx reabsorption (acral osteolysis).
Fingernail capillary bed demonstrating capillary dropout with large dilated vessels.
Lung biopsy demonstrating severe interstitial fibrosis and medial fibrosis and smooth muscle hyperplasia of a pulmonary arteriole compatible with pulmonary hypertension.
Lung biopsy demonstrating expansion of the interstitium of the lung by fibrous tissue along with chronic inflammatory cells.
Barium swallow demonstrating reflux into the distal esophagus, as well as an accordion appearance in the duodenum.
Skin biopsy showing extensive fibrosis. The biopsy has a square morphology, which reflects the rigidity of the tissue biopsy specimen due to striking pan-dermal sclerosis. In addition, the fibrosing reaction extends into the panniculus. The number of adnexal structures is reduced, another characteristic feature of scleroderma. A significant inflammatory cell infiltrate is not observed. This is in contradistinction to morphea, in which a prominent inflammatory cell infiltrate is present.
Skin biopsy showing severe fibrosis. The fibrosis reflects a widening of collagen bundles in concert with an increase in the number of collagen fibers. Note the superimposed deposition of the newly synthesized delicate collagen bundles interposed between the preexisting collagen bundles, the latter appearing wide and manifesting a hyalinized morphology.
 
 
 
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