Abstract
Juvenile systemic sclerosis is an orphan disease of the macro- and microvasculature with an unknown etiology. Although pediatric and adult classification systems have been developed, neither has been validated yet. Recent publications have shown a shift in the subset pattern in juvenile-onset adult patients, with a better outcome in these patients as compared with young adult patients. As a first step in assessing activity, damage, and severity, a pediatric severity score—a modification of the adult Medsger scale—was proposed but has not been validated. An activity score also has been developed but has not yet been published. Currently, treatment approaches are based only on adult data, and problems exist in extrapolating these data to a pediatric population.
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Herrick AL, Ennis H, Bhushan M, Silman AJ, Baildam EM. Incidence of childhood linear scleroderma and systemic sclerosis in the UK and Ireland. Arthritis Care Res (Hoboken). 2010;62(2):213–8.
Matucci-Cerinic M, Kahaleh B, Wigley FM. Review: evidence that systemic sclerosis is a vascular disease. Arthritis Rheum. 2013;65(8):1953–62.
Minier T, Guiducci S, Bellando-Randone S, Bruni C, Lepri G, Czirjak L, et al. Preliminary analysis of the very early diagnosis of systemic sclerosis (VEDOSS) EUSTAR multicentre study: evidence for puffy fingers as a pivotal sign for suspicion of systemic sclerosis. Ann Rheum Dis. 2014;73(12):2087–93.
Kahaleh MB. Raynaud phenomenon and the vascular disease in scleroderma. Curr Opin Rheumatol. 2004;16(6):718–22.
Kahaleh B, Mulligan-Kehoe MJ. Mechanism of vascular disease. In: Varga J, Denton C, Wigley F, editors. Scleroderma: from pathogenesis to comprehensive management. London: Springer; 2012. p. 227–46.
Taylor MH, McFadden JA, Bolster MB, Silver RM. Ulnar artery involvement in systemic sclerosis (scleroderma). J Rheumatol. 2002;29(1):102–6.
Rosato E, Gigante A, Barbano B, Cianci R, Molinaro I, Pisarri S, et al. In systemic sclerosis macrovascular damage of hands digital arteries correlates with microvascular damage. Microvasc Res. 2011;82(3):410–5.
Rajkumar VS, Sundberg C, Abraham DJ, Rubin K, Black CM. Activation of microvascular pericytes in autoimmune Raynaud’s phenomenon and systemic sclerosis. Arthritis Rheum. 1999;42(5):930–41.
Reiff A, Weinberg KI, Triche T, Masinsin B, Mahadeo KM, Lin CH, et al. T lymphocyte abnormalities in juvenile systemic sclerosis patients. Clin Immunol. 2013;149(1):146–55.
Zulian F, Woo P, Athreya BH, Laxer RM, Medsger Jr TA, Lehman TJ, et al. The Pediatric Rheumatology European Society/American College of Rheumatology/European League against Rheumatism provisional classification criteria for juvenile systemic sclerosis. Arthritis Rheum. 2007;57(2):203–12.
van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, et al. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis. 2013;72(11):1747–55.
Foeldvari I, Zhavania M, Birdi N, Cuttica RJ, de Oliveira SH, Dent PB, et al. Favourable outcome in 135 children with juvenile systemic sclerosis: results of a multi-national survey. Rheumatology (Oxford). 2000;39(5):556–9.
Martini G, Foeldvari I, Russo R, Cuttica R, Eberhard A, Ravelli A, et al. Systemic sclerosis in childhood: clinical and immunologic features of 153 patients in an international database. Arthritis Rheum. 2006;54(12):3971–8.
Foeldvari I, Nihtyanova SI, Wierk A, Denton CP. Characteristics of patients with juvenile onset systemic sclerosis in an adult single-center cohort. J Rheumatol. 2010;37:2422–6.
Foeldvari I, Tyndall A, Zulian F, Muller-Ladner U, Czirjak L, Denton C, et al. Juvenile and young adult-onset systemic sclerosis share the same organ involvement in adulthood: data from the EUSTAR database. Rheumatology (Oxford). 2012;51:1832–7.
LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger Jr TA, et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol. 1988;15(2):202–5.
Merkel PA, Silliman NP, Clements PJ, Denton CP, Furst DE, Mayes MD, et al. Patterns and predictors of change in outcome measures in clinical trials in scleroderma: an individual patient meta-analysis of 629 subjects with diffuse cutaneous systemic sclerosis. Arthritis Rheum. 2012;64(10):3420–9.
Medsger Jr TA, Silman AJ, Steen VD, Black CM, Akesson A, Bacon PA, et al. A disease severity scale for systemic sclerosis: development and testing. J Rheumatol. 1999;26(10):2159–67.
Hudson M, Steele R, Baron M. Update on indices of disease activity in systemic sclerosis. Semin Arthritis Rheum. 2007;37(2):93–8.
Vanthuyne M, Smith V, De Langhe E, Van Praet J, Arat S, Depresseux G, et al. The Belgian Systemic Sclerosis Cohort: correlations between disease severity scores, cutaneous subsets, and autoantibody profile. J Rheumatol. 2012;39(11):2127–33.
La Torre F, Martini G, Russo R, Katsicas MM, Corona F, Calcagno G, et al. A preliminary disease severity score for juvenile systemic sclerosis. Arthritis Rheum. 2012;64(12):4143–50.
Foeldvari I, Wierk A. Healthy children have a significantly increased skin score assessed with the modified Rodnan skin score. Rheumatology. 2006;45:76–8.
Rajaram S, Swift AJ, Capener D, Elliot CA, Condliffe R, Davies C, et al. Comparison of the diagnostic utility of cardiac magnetic resonance imaging, computed tomography, and echocardiography in assessment of suspected pulmonary arterial hypertension in patients with connective tissue disease. J Rheumatol. 2012;39(6):1265–74.
Khanna D, Distler O, Avouac J, Behrens F, Clements PJ, Denton C, et al. Measures of response in clinical trials of systemic sclerosis: the combined response index for systemic sclerosis (CRISS) and Outcome Measures in Pulmonary Arterial Hypertension related to Systemic Sclerosis (EPOSS). J Rheumatol. 2009;36(10):2356–61.
Khanna D, Lovell DJ, Giannini E, Clements PJ, Merkel PA, Seibold JR, et al. Development of a provisional core set of response measures for clinical trials of systemic sclerosis. Ann Rheum Dis. 2008;67(5):703–9.
Castelino FV, Varga J. Current status of systemic sclerosis biomarkers: applications for diagnosis, management and drug development. Expert Rev Clin Immunol. 2013;9(11):1077–90.
van Bon L, Affandi AJ, Broen J, Christmann RB, Marijnissen RJ, Stawski L, et al. Proteome-wide analysis and CXCL4 as a biomarker in systemic sclerosis. N Engl J Med. 2014;370(5):433–43.
Rimar D, Rosner I, Nov Y, Slobodin G, Rozenbaum M, Halasz K, et al. Brief report: lysyl oxidase is a potential biomarker of fibrosis in systemic sclerosis. Arthritis Rheumatol. 2014;66(3):726–30.
Ingegnoli F, Gualtierotti R, Schioppo T, Orenti A, Boracchi P, Lubatti C, et al. Fibrosis biomarkers in isolated Raynaud’s phenomenon: too little, too soon? Ann Rheum Dis. 2013.
Muangchan C, Harding S, Khimdas S, Bonner A, Canadian Scleroderma Research Group, Baron M, et al. Association of C-reactive protein with high disease activity in systemic sclerosis: results from the Canadian Scleroderma Research Group. Arthritis Care Res (Hoboken). 2012;64(9):1405–14.
Schwaiger JP, Khanna D, Gerry CJ. Screening patients with scleroderma for pulmonary arterial hypertension and implications for other at-risk populations. Eur Res Rev: Off J Eur Res Soc. 2013;22(130):515–25.
Bonella F, Volpe A, Caramaschi P, Nava C, Ferrari P, Schenk K, et al. Surfactant protein D and KL-6 serum levels in systemic sclerosis: correlation with lung and systemic involvement. Sarcoidosis Vasc Diffuse Lung Dis. 2011;28(1):27–33.
Cappelli S, Bellando-Randone S, Guiducci S, Matucci-Cerinic M. Is immunosuppressive therapy the anchor treatment to achieve remission in systemic sclerosis? Rheumatology (Oxford). 2014;53(6):975–87.
Poormoghim H, Moradi Lakeh M, Mohammadipour M, Sodagari F, Toofaninjed N. Cyclophosphamide for scleroderma lung disease: a systematic review and meta-analysis. Rheumatol Int. 2012;32(8):2431–44.
van Laar JM, Farge D, Sont JK, Naraghi K, Marjanovic Z, Larghero J, et al. Autologous hematopoietic stem cell transplantation vs intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis: a randomized clinical trial. JAMA. 2014;311(24):2490–8.
Elhai M, Meunier M, Matucci-Cerinic M, Maurer B, Riemekasten G, Leturcq T, et al. Outcomes of patients with systemic sclerosis-associated polyarthritis and myopathy treated with tocilizumab or abatacept: a EUSTAR observational study. Ann Rheum Dis. 2013;72(7):1217–20.
Daoussis D, Liossis SN, Tsamandas AC, Kalogeropoulou C, Paliogianni F, Sirinian C, et al. Effect of long-term treatment with rituximab on pulmonary function and skin fibrosis in patients with diffuse systemic sclerosis. Clin Exp Rheumatol. 2012;30(2 Suppl 71):S17–22.
Topal AA, Dhurat RS. Scleroderma therapy: clinical overview of current trends and future perspective. Rheumatol Int. 2013;33(1):1–18.
Walker KM, Pope J. Treatment of systemic sclerosis complications: what to use when first-line treatment fails–a consensus of systemic sclerosis experts. Semin Arthritis Rheum. 2012;42(1):42–55.
Kowal-Bielecka O, Landewe R, Avouac J, Chwiesko S, Miniati I, Czirjak L, et al. EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR). Ann Rheum Dis. 2009;68(5):620–8.
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Ivan Foeldvari declares the receipt of consulting fees from Chugai, Abbvie, Pfizer, and SOBI.
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Foeldvari, I. Update on Juvenile Systemic Sclerosis. Curr Rheumatol Rep 17, 18 (2015). https://doi.org/10.1007/s11926-014-0491-y
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DOI: https://doi.org/10.1007/s11926-014-0491-y