Current Developments in Pediatric Systemic Sclerosis

Overview

Journal Curr Rheumatol Rep

Publisher Current Science

Specialty Rheumatology

Date 2009 Mar 20

PMID 19296881

Citations 1

Authors

Ivan Foeldvari

Affiliations

Soon will be listed here.

Abstract

Juvenile systemic sclerosis is an orphan disease with an incidence of around 0.05 per 100,000 children. The mean age of onset is approximately 8 years, and 90% of pediatric patients have a diffuse subset. The organ involvement of pediatric patients differs from adult patients. Survival rates are significantly better in pediatric patients than in adult patients. Most patients who die in the first 5 years of the disease have a diffuse subset. Interestingly, the disease subset shifts when pediatric patients are reviewed in adult cohorts of systemic sclerosis patients, in which only around 40% of patients have diffuse subset. The pediatric-onset patients still have a low prevalence of anticentromere antibodies. There is a larger group of patients with overlap features in juvenile systemic sclerosis than in adult cohorts. There are also noticeable differences in organ involvement.

Citing Articles

Interstitial lung diseases in children.

Clement A, Nathan N, Epaud R, Fauroux B, Corvol H Orphanet J Rare Dis. 2010; 5:22.

PMID: 20727133 PMC: 2939531. DOI: 10.1186/1750-1172-5-22.

References

Russo R, Katsicas M . Clinical characteristics of children with Juvenile Systemic Sclerosis: follow-up of 23 patients in a single tertiary center. Pediatr Rheumatol Online J. 2007; 5:6. PMC: 1869029. DOI: 10.1186/1546-0096-5-6. View

Vesely R, Vargova V, Ravelli A, Massa M, Oleksak E, DAlterio R . Serum level of KL-6 as a marker of interstitial lung disease in patients with juvenile systemic sclerosis. J Rheumatol. 2004; 31(4):795-800. View

LeRoy E, Black C, FLEISCHMAJER R, Jablonska S, Krieg T, Medsger Jr T . Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol. 1988; 15(2):202-5. View

Aoyama K, Nagai Y, Endo Y, Ishikawa O . Juvenile systemic sclerosis: report of three cases and review of Japanese published work. J Dermatol. 2007; 34(9):658-61. DOI: 10.1111/j.1346-8138.2007.00350.x. View

Varga J, Abraham D . Systemic sclerosis: a prototypic multisystem fibrotic disorder. J Clin Invest. 2007; 117(3):557-67. PMC: 1804347. DOI: 10.1172/JCI31139. View

Pelkonen P, Jalanko H, Lantto R, Makela A, Pietikainen M, Savolainen H . Incidence of systemic connective tissue diseases in children: a nationwide prospective study in Finland. J Rheumatol. 1994; 21(11):2143-6. View

Zulian F, Woo P, Athreya B, Laxer R, Medsger Jr T, Lehman T . 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. DOI: 10.1002/art.22551. View

Foeldvari I, Zhavania M, Birdi N, Cuttica R, de Oliveira S, DENT P . Favourable outcome in 135 children with juvenile systemic sclerosis: results of a multi-national survey. Rheumatology (Oxford). 2000; 39(5):556-9. DOI: 10.1093/rheumatology/39.5.556. View

LeRoy E, Medsger Jr T . Criteria for the classification of early systemic sclerosis. J Rheumatol. 2001; 28(7):1573-6. View

10.

Scalapino K, Arkachaisri T, Lucas M, Fertig N, Helfrich D, Londino Jr A . Childhood onset systemic sclerosis: classification, clinical and serologic features, and survival in comparison with adult onset disease. J Rheumatol. 2006; 33(5):1004-13. View

11.

Martini G, Foeldvari I, Russo R, Cuttica R, Eberhard A, Ravelli A . Systemic sclerosis in childhood: clinical and immunologic features of 153 patients in an international database. Arthritis Rheum. 2006; 54(12):3971-8. DOI: 10.1002/art.22207. View

12.

Arias-Nunez M, Llorca J, Vazquez-Rodriguez T, Gomez-Acebo I, Miranda-Filloy J, Martin J . Systemic sclerosis in northwestern Spain: a 19-year epidemiologic study. Medicine (Baltimore). 2008; 87(5):272-280. DOI: 10.1097/MD.0b013e318189372f. View

13.

Walker U, Tyndall A, Czirjak L, Denton C, Farge-Bancel D, Kowal-Bielecka O . Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials And Research group database. Ann Rheum Dis. 2007; 66(6):754-63. PMC: 1954657. DOI: 10.1136/ard.2006.062901. View

14.

Foeldvari I, Wierk A . Healthy children have a significantly increased skin score assessed with the modified Rodnan skin score. Rheumatology (Oxford). 2005; 45(1):76-8. DOI: 10.1093/rheumatology/kei106. View

15.

Misra R, Singh G, Aggarwal P, Aggarwal A . Juvenile onset systemic sclerosis: a single center experience of 23 cases from Asia. Clin Rheumatol. 2006; 26(8):1259-62. DOI: 10.1007/s10067-006-0483-z. View

16.

Martini G, Vittadello F, Kasapcopur O, Magni Manzoni S, Corona F, Duarte-Salazar C . Factors affecting survival in juvenile systemic sclerosis. Rheumatology (Oxford). 2008; 48(2):119-22. DOI: 10.1093/rheumatology/ken388. View

17.

Dolezalova P, Young S, Bacon P, Southwood T . Nailfold capillary microscopy in healthy children and in childhood rheumatic diseases: a prospective single blind observational study. Ann Rheum Dis. 2003; 62(5):444-9. PMC: 1754542. DOI: 10.1136/ard.62.5.444. View

18.

Mayes M, Trojanowska M . Genetic factors in systemic sclerosis. Arthritis Res Ther. 2007; 9 Suppl 2:S5. PMC: 2072883. DOI: 10.1186/ar2189. View

19.

Gabrielli A, Svegliati S, Moroncini G, Avvedimento E . Pathogenic autoantibodies in systemic sclerosis. Curr Opin Immunol. 2007; 19(6):640-5. DOI: 10.1016/j.coi.2007.11.004. View

20.

Czirjak L, Kumanovics G, Varju C, Nagy Z, Pakozdi A, Szekanecz Z . Survival and causes of death in 366 Hungarian patients with systemic sclerosis. Ann Rheum Dis. 2007; 67(1):59-63. DOI: 10.1136/ard.2006.066340. View