The aim of this study was to describe the clinical manifestations and outcomes of a national cohort of childhood systemic lupus erythematosus (cSLE). All cases of cSLE registered in the Israeli national registry of children with rheumatic diseases between 1987–2003 were examined for disease activity and damage by the SLE disease activity index (SLEDAI) and SLE collaborating clinics/American College of Rheumatology (SLICC/ACR) damage index. Demographic, clinical, laboratory and treatment factors were analysed for their effect on the outcome. One-hundred and two patients were identified, 81% females, with a mean age at diagnosis of 13.3 ± 2.6 years. The mean SLEDAI score was 17.2 ± 9.0 (range 2–60). Fifty four patients were followed for at least five years. The mean SLEDAI decreased to 7.6 ± 6.3 (0–29) and the mean SLICC/ACR damage index was 0.7 ± 1.6 (0–8). Five patients developed chronic renal failure. No patients died. No factors were found to be significantly associated with the outcome except the initial SLEDAI score. The five-year outcome of our national cSLE cohort was good; with relatively low activity and minimal damage in most patients. The initial SLEDAI predicted the development of late damage.
Fessel WJ. Epidemiology of systemic lupus erythematosus. Rheum Dis Clin North Am1988; 14: 15–23.
3.
Kaufman DB, Laxer RM, Silverman ED, Stein L. Systemic lupus erythematosus in childhood and adolescence – the problem, epidemiology, incidence, susceptibility, genetics, and prognosis. Curr Prob Pediatr1986; 16: 555–624.
4.
Tucker LB, Menon S, Schaller JG, Isenberg DA. Adult and childhood-onset systemic lupus erythematosus: a comparison of onset, clinical features, serology and outcome. Br J Rheumatol1995; 34: 866–872.
5.
Carreno L, Lopez-Longo FJ, Monteagudo I, Rodriguez-Mahou M, Bascones M, Gonzalez CM. Immunological and clinical differences between juvenile and adult onset of systemic lupus erythematosus. Lupus1999; 8: 287–292.
6.
Font J, Cervera R, Espinosa G, Pallares L, Ramos-Casals M, Jimenez S. Systemic lupus erythematosus (SLE) in childhood: analysis of clinical and immunological findings in 34 patients and comparison with SLE characteristics in adults. Ann Rheum Dis1998; 57: 456–459.
7.
Costallat LTL, Coimbra AMV. Systemic lupus erythematosus: clinical and laboratory aspects related to age at disease onset. Clin Exp Rheumatol1994; 12: 603–607.
8.
Brunner HI, Silverman ED, To T, Bombardier C, Feldman BM. Risk factors for damage in childhood-onset systemic lupus erythematosus. Arthritis Rheum2002; 46: 436–444.
9.
Font J, Cervera R, Ramos-Casals M et al. Clusters of clinical and immunologic features in systemic lupus erythematosus: analysis of 600 patients from a single center. Semin Arthritis Rheum2004; 33: 217–230.
10.
Rood MJ, ten Cate R, Van Suijlekom-Smit LWA, den Ouden EJ, Ouwerkerk FE, Breedveld FC. Childhood-onset systemic lupus erythematosus. Scand J Rheumatol1999; 28: 222–226.
11.
Ravelli A, Duarte-Salazar C, Buratti S et al. Assessment of damage in juvenile-onset systemic lupus erythematosus: a multicenter cohort study. Arthritis Rheum2003; 49: 501–507.
12.
Ruperto N, Ravelli A, Cuttica R et al. The Pediatric Rheumatology International Trials Organization criteria for the evaluation of response to therapy in juvenile systemic lupus erythematosus: Prospective validation of the disease activity core set. Arthritis Rheum2005; 52: 2854–2864.
13.
Bader-Meunier B, Armengaud JB, Haddad E et al. Initial presentation of childhood-onset systemic lupus erythematosus: a French multicenter study. J Pediatr2005; 146: 648–653.
14.
Alsaeid K, Kamal H, Haider MZ, Al-Enezi HM, Malaviya AN. Systemic lupus erythematosus in Kuwaiti children: organ system involvement and serological findings. Lupus2004; 13: 613–617.
15.
Cassidy JT, Sullivan DB, Petty RE et al. Lupus nephritis and encephalopathy: prognosis in 58 children. Arthritis Rheum1977; 20: 287–293.
Lehman TJA, McCurdy D, Bernstein BH et al. Systemic lupus erythematosus in the first decade of life. Pediatrics1989; 83: 235–239.
18.
King KK, Korenrich HK, Bernstein BH et al. The clinical spectrum of systemic lupus erythematosus in childhood. Arthritis Rheum1977; 20: 287–294.
19.
Brik R, Padeh S, Mukamel M et al. Systemic lupus erythematosus in children in Israel. Harefuah1995; 129: 233–235.
20.
Uziel Y, Hashkes PJ. The state of pediatric rheumatology in Israel. PROJ2004; 2: 172–175 (electronic: http://www.pedrheumonlinejournal.org/).
21.
Hashkes PJ, Uziel Y, Rubenstein M et al. The Israeli internet-based pediatric rheumatology registry: novel concept of multicenter data collection in pediatric rheumatology. PROJ2004; 2: 37–50 (electronic: http://www.pedrheumonlinejournal.org/).
22.
Tan EM, Cohen AS, Fries JF et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum1982; 25: 1271–1277.
23.
Bombardier C, Gladman DD, Urowitz MB, Caron D, Chang CH. Derivation of the SLEDAI: a disease activity index for lupus patients. The committee on prognosis in SLE. Arthritis Rheum1992; 35: 630–640.
24.
Brunner HI, Feldman BM, Bombardier C, Silverman ED. Sensitivity of the systemic lupus erythematosus disease activity index, British, Isles lupus assessment group index, and systemic lupus activity measure in the evaluation of clinical change in childhood-onset systemic lupus erythematosus. Arthritis Rheum1999; 42: 1354–1360.
25.
Gladman D, Ginzler E, Goldsmith C et al. The development and initial validation of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index for systemic lupus erythematosus. Arthritis Rheum1996; 39: 363–369.
26.
Malleson PN, Fung MY, Rosenberg AM. The incidence of pediatric rheumatic diseases: Result from the Canadian Pediatric Rheumatology Association Disease Registry. J Rheumatol1996; 23: 1981–1987.
27.
Kaipiainen-Sappanen O, Savolainen A. Incidence of chronic juvenile rheumatic diseases in Finland during 1980–1990. Clin Exp Rheumatol1996; 14: 441–444.
28.
Cameron JS. Lupus nephritis in childhood and adolescence. Pediatr Nephrol1994; 8: 230–249.
29.
Parikh S, Swaiman KF, Kim Y. Neurologic characteristics of childhood lupus erythematosus. Pediatr Neurol1995; 13: 198–201.
Seaman DE, Londino AV Jr, Kwoh CK, Medsger TA Jr, Manzi S. Antiphospholipid antibodies in pediatric systemic lupus erythematosus. Pediatrics1995; 96: 1040–1045.
33.
Petri M. Epidemiology of the antiphospholipid antibody syndrome; J Autoimmun2000; 15: 145–151.
