Abstract
Systemic sclerosis (SSc) is a chronic disease of unknown etiology, and its classification as a systemic autoimmune rheumatic disease (SARD) is supported by clinical and experimental observations that include the presence of autoantibodies (AAs) and autoreactive T cells. Although some AAs are specific markers for the disease, there has not been widespread adoption of them as specific classification criteria. The application of classification criteria is further hampered by the recognition that diagnosis of SSc encompasses a wide variety of clinical features that prompts the classification of the condition into a number of disease subsets. For practical purposes, most clinicians subclassify the disease as limited cutaneous SSc (lcSSc) or diffuse cutaneous SSc (dcSSc). As diagnostic technologies rapidly evolve and clinicians consider designer therapies for this condition, the development and universal adoption of clinical classification criteria will be a significant challenge.
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References
Walker JG, Pope J, Baron M, Leclercq S, Hudson M, Taillefer S, Edworthy SM, Nadashkevich O, Fritzler MJ. The development of systemic sclerosis classification criteria. Clin Rheumatol 2007; 26: 1401–1409.
Steen VD. Clinical manifestations of systemic sclerosis. Semin Cutan Med Surg 1998; 17: 48–54.
Mayes MD, Trojanowska M. Genetic factors in systemic sclerosis. Arthritis Res Ther 2007; 9 Suppl 2: S5.
Valentini G, Della Rossa A, Bombardieri S, Bencivelli W, Silman AJ, D’Angelo S, Cerinic MM, Belch JF, Black CM, Bruhlmann P, Czirják L, De Luca A, Drosos AA, Ferri C, Gabrielli A, Giacomelli R, Hayem G, Inanc M, McHugh NJ, Nielsen H, Rosada M, Scorza R, Stork J, Sysa A. European multicentre study to define disease activity criteria for systemic sclerosis. II. Identification of disease activity variables and development of preliminary activity indexes. Ann Rheum Dis 2001; 60: 592–598.
Walker JG, Fritzler MJ. Update on autoantibodies in systemic sclerosis. Curr Opinion Rheum 2007; 19: 580–591.
Santiago M, Baron M, Hudson M, Burlingame RW, The Canadian Scleroderma Research Group, Fritzler MJ. Antibodies to RNA polymerase III in systemic sclerosis as detected by an ELISA. J Rheumatol 2007; 34: 1528–1534.
Mahler M, Raijmakers R. Novel aspects of autoantibodies to the PM/Scl complex: clinical, genetic and diagnostic insights. Autoimmun Rev 2007; 6: 432–437.
Baroni SS, Santillo M, Bevilacqua F, Luchetti M, Spadoni T, Mancini M, Fraticelli P, Sambo P, Funaro A, Kazlauskas A, Avvedimento EV, Gabrielli A. Stimulatory autoantibodies to the PDGF receptor in systemic sclerosis. N Engl J Med 354: 2667–2676.
Walker JG, Barnabe C, Fritzler MJ. Autoantibodies, classification criteria and diagnosis of systemic autoimmune rheumatic diseases. In: Conrad K, Chan EKL, Fritzler MJ, Sack U, Shoenfeld Y, Wiik A (eds): From Etiopathogenesis to the Prediction of Autoimmune Diseases: Relevance of Autoantibodies. Lengerich, Germany, Pabst Science Publishers, 2007, pp. 352–369.
Masi AT, Rodnan GP, Medsger T, Jr., Altman RD, D’Angelo WA, Fries JF, LeRoy EC, Kirsner AB, Mackenzie AH, McShane DJ, Myers AR, Sharp GC. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 1980; 23: 581–590.
Lonzetti LS, Joyal F, Raynauld JP, Roussin A, Goulet JR, Rich E, Choquette D, Raymond Y, Senécal JL. 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 2001; 44: 735–736.
Nadashkevich O, Davis P, Fritzler MJ. A proposal of criteria for the classification of systemic sclerosis. Medical Science Monitor 2004; 10: 615–621.
Johnson SR, Feldman BM, Hawker GA. Classification criteria for Systemic Sclerosis subsets. J Rheumatol 2007; 34: 1855–1863.
LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA, Rowell N, Wollheim F. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 1988; 15: 202–205.
LeRoy EC, Medsger TA, Jr. Criteria for the classification of early systemic sclerosis. J Rheumatol 2001; 28: 1573–1576.
Maricq HR, Valter I. A working classification of scleroderma spectrum disorders: A proposal and the results of testing on a sample of patients. Clin Exp Rheumatol 2004; 22: S5–S13.
Fritzler MJ. Advances and applications of multiplexed diagnostic technologies in autoimmune diseases. Lupus 2006; 15: 422–427.
Dazzi F, van Laar JM, Cope A, Tyndall A. Cell therapy for autoimmune diseases. Arthritis Res Ther 2007; 9: 206.
Denton CP. Therapeutic targets in systemic sclerosis. Arthritis Res Ther 2007; 9 Suppl. 2: S6.
Bissonnette L, Bergeron MG. Next revolution in the molecular theranostics of infectious diseases: microfabricated systems for personalized medicine. Expert Rev Mol Diagn 2006; 6: 433–450.
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© 2008 Humana Press, a part of Springer Science+Business Media, LLC
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Walker, J.G., Fritzler, M.J. (2008). Systemic Sclerosis. In: Shoenfeld, Y., Cervera, R., Gershwin, M.E. (eds) Diagnostic Criteria in Autoimmune Diseases. Humana Press. https://doi.org/10.1007/978-1-60327-285-8_6
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DOI: https://doi.org/10.1007/978-1-60327-285-8_6
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