Advertisement

Clinical Reviews in Allergy & Immunology

, Volume 40, Issue 2, pp 78–83 | Cite as

Diagnosis and Classification of Systemic Sclerosis

  • Eric HachullaEmail author
  • David Launay
Article

Abstract

As the diagnosis of systemic sclerosis (SSc) is generally suggested by the presence of Raynaud’s phenomenon followed by typical skin thickening associated with the presence of additional extracutaneous features, capillaroscopic abnormalities, and characteristic autoantibodies, the first classification criteria, published by the American Rheumatism Association in 1980, were based only on clinical and chest X-ray items. As a consequence, 10% to 20% of the patients did not meet these criteria. In 1988, an international consensus was reached resulting in the proposal of a new and more practical classification based on the judgment and clinical practice of an expert panel. This classification introduced the SSc nail fold capillaroscopy abnormalities (dilation and/or avascular areas) and specific antinuclear antibodies. Two subsets of SSc emerged from discussions: diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc). The calcifications, Raynaud’s phenomenon, esophageal hypomotility, sclerodactyly, and telangiectasia (CREST) syndrome can be considered an lcSSc. In 2001, LeRoy and Medsger, realizing the shortcomings of the 1988 subsets in being too exclusive and taking advantage of increased experience with nail fold capillaroscopy and autoantibody determination, proposed criteria for an additional early or limited subset of SSc (lSSc), to supplement the previously recognized lcSSc and dcSSc forms. Patients with lSSc must have Raynaud’s phenomenon and SSc-specific nail fold capillary changes and/or SSc-specific autoantibodies. Some lSSc patients who have no cutaneous involvement but common SSc nail fold capillaroscopy abnormalities, specific antinuclear antibodies, and visceral involvement are sometimes called SSc sine scleroderma. Whether or not lSSc and SSc sine scleroderma are the same or two different subsets is currently not known.

Keywords

Systemic sclerosis Scleroderma Diagnosis Classification 

References

  1. 1.
    Mayes MD, Lacey JV Jr, Beebe-Dimmer J et al (2003) Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum 48:2246–2255CrossRefPubMedGoogle Scholar
  2. 2.
    Le Guern V, Mahr A, Mouthon L, Jeanneret D, Carzon M, Guillevin L (2004) Prevalence of systemic sclerosis in a French multi-ethnic county. Rheumatology (Oxford) 43:1129–1137CrossRefGoogle Scholar
  3. 3.
    Scussel-Lonzetti L, Joyal F, Raynauld JP et al (2002) Predicting mortality in systemic sclerosis: analysis of a cohort of 309 off French Canadian patients with emphasis on features at diagnosis as predictive factors for survival. Medicine (Baltimore) 81:154–167CrossRefGoogle Scholar
  4. 4.
    Steen VD, Medsger TA (2007) Changes in causes of death in systemic sclerosis, 1972-2002. Ann Rheum Dis 66:940–944CrossRefPubMedGoogle Scholar
  5. 5.
    Vonk MC, Broers B, Heijdra YF et al (2008) Systemic sclerosis and its pulmonary complications in The Netherlands: an epidemiological study. Ann Rheum Dis 68:961–965CrossRefPubMedGoogle Scholar
  6. 6.
    Reveille JD, Solomon DH, American College of Rheumatology Ad Hoc Committee of Immunologic Testing Guidelines (2003) Evidence-based guidelines for the use of immunologic tests: anticentromere, Scl-70, and nucleolar antibodies. Arthritis Rheum 49:399–412CrossRefPubMedGoogle Scholar
  7. 7.
    Hunzelmann N, Genth E, Krieg T et al (2008) The registry of the German Network for Systemic Scleroderma: frequency of disease subsets and patterns of organ involvement. Rheumatology 47:1185–1192CrossRefPubMedGoogle Scholar
  8. 8.
    Johnson SR, Feldman BM, Hawker GA (2007) Classification criteria for systemic sclerosis subsets. J Rheumatol 34:1855–1863PubMedGoogle Scholar
  9. 9.
    Masy AT, Rodnan GP, Medsger TA Jr, Altman RD, D’angelo WA, Fries JF, the Subcommittee for Scleroderma Criteria of American Rheumatism Association Diagnostic and Therapeutic Criteria Committee (1980) Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 23:581–590CrossRefGoogle Scholar
  10. 10.
    Medsger TA Jr (1985) Comment on Scleroderma Criteria Cooperative Study. In: Black CM, Myers AR (eds) Current topics in rheumatology: systemic sclerosis (scleroderma). Gower Medical Publishing, New York, pp 16–17Google Scholar
  11. 11.
    Lonzetti LS, Joyal F, Raynauld JP et al (2001) Updating the American College of Rheumatology preliminary classification criteria for systemic sclerosis: addition of severe nailfold capillaroscopy abnormalities markedly increases the sensitivity for limited scleroderma. Arthritis Rheum 44:735–736CrossRefPubMedGoogle Scholar
  12. 12.
    Meyer OC, Fertig N, Lucas M, Somogyi N, Medsger TA Jr (2007) Disease subsets, antinuclear antibody profile, and clinical features in 127 French and 247 US adult patients with systemic sclerosis. J Rheumatol 34:104–109PubMedGoogle Scholar
  13. 13.
    LeRoy EC, Black C, Fleischmajer R et al (1988) Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 15:202–205PubMedGoogle Scholar
  14. 14.
    Medsger TA Jr (2003) Natural history of systemic sclerosis and assessment of disease activity, severity, functional status, and psychologic well-being. Rhum dis Clin N Am 29:255–273CrossRefGoogle Scholar
  15. 15.
    Penn H, Howie AJ, Kingdon EJ, Bunn CCRJ et al (2007) Scleroderma renal crisis: patient characteristics and long-term outcomes. Q J Med 100:485–494Google Scholar
  16. 16.
    Winterbauer RH (1964) Multiple telangiectasia, Raynaud’s phenomenon, sclerodactyly, and subcutaneous calcinosis: a syndrome mimicking hereditary hemorrhagic telangiectasia. Bull Johns Hopkins Hosp 114:361–383PubMedGoogle Scholar
  17. 17.
    Barnett AJ, Miller M, Littlejohn GO (1988) The diagnosis and classification of scleroderma (systemic sclerosis). Postgrad Med J 64:121–125CrossRefPubMedGoogle Scholar
  18. 18.
    LeRoy EC, Medsger TA Jr (2001) Criteria for the classification of early systemic sclerosis. J Rheumatol 28:1573–1576PubMedGoogle Scholar
  19. 19.
    Poormoghim H, Lucas M, Fertig N, Medsger TA Jr (2000) Systemic sclerosis sine scleroderma. Demographic, clinical, and serological features and survival in forty-eight patients. Arthritis Rheum 43:444–451CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  1. 1.Department of Internal Medicine, National Scleroderma CenterHôpital Claude Huriez, Université de Lille 2Lille cedexFrance

Personalised recommendations