Abstract
Objective: To analyze clinical, laboratory and treatment features associated with death in a childhood-onset SLE population. Patients and methods: Patients with childhood-onset SLE followed at the State University of Campinas, Brazil, between 1980 and 2002 were included. Data on clinical and laboratory features of the disease were collected regularly. Logistic regression was used for analyzing association between clinical and laboratory features and death. Kaplan–Meyer tests were used to estimate the survival curves. Results: Of 61 patients identified, six were lost to follow-up during the first year of disease. The mean follow-up period of the remaining 55 patients was 3.25 years (SD=1.2). Mean SLICC/ACR-DI score was 4.9 (SD=3.4). Death occurred in 12 (21.8%) of 55 patients. Direct causes of death were: infection in six (50%), stroke in four (33.3%), and renal insufficiency in two (16.7%). Five patients (41.7%) died during the first 5 years of disease due to infection. Male gender (p=0.004; OR=9.1; 95% CI=7.6–21.0), infection (p=0.001; OR=4.2; 95% CI=1.6–15.2) and nephritis (p=0.02; OR=2.3; 95% CI=1.3–5.2) were independent factors associated with death in the multivariate analysis. The global survival rate adjusted for duration of disease was 93.9% in the first year of disease, 88.9% in the second year, 80.8% in the fifth year and 48.1% in 20 years of follow-up. When comparing survival curves, male gender, the presence of infection during the course of the disease and the presence of nephritis during follow-up had a worse survival. Conclusion: Male gender, the presence of infection and nephritis were independent risk factors for death in our Brazilian cohort. Damage did not independently influence survival in this study.
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References
Cervera R, Khamashta MA, Font J, Sebastiani GD, Gil A, Lavilla P, Domenech I et al (1993) Systemic lupus erythematosus: clinical and immunological patterns of disease expression in a cohort of 1000 patients. Medicine 72:113–124
Nepom BS, Schaller JG (1984) Childhood systemic lupus erythematosus. Prog Clin Rheumatol 1:33–69
Schaller J (1982) Lupus in childhood. Clin Rheum Dis 8:219–228
Rosenberg AM (1994) Systemic lupus erythematosus in children. Springer Semin Immunopathol 16:216–279
Costallat LTL, Coimbra AMV (1994) Systemic lupus erythematosus: clinical and laboratory aspects to age at disease onset. Clin Experim Rheumatol 12:603–607
Font J, Cervera R, Espinosa G, Pallares L, Ramos-Casals M, Jimenez S et al (1998) Systemic lupus erythematosus (SLE) in childhood: analysis of clinical and immunological findings in 34 patients and comparison with SLE characteristics in adults. Ann Rheum Dis 57:456–459
Hashimoto H, Hiroshi T, Hirano T (1987) Differences in clinical and immunological findings of systemic lupus erythematosus related to age. J Rheumatol 14:497–501
Tan EM, Cohen AS, Fries JF et al (1982) The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 25:1271–1277
Liang MH, Corzillius M, Bae SC et al (1999) The American college nomenclature and case definitions for neuropsychiatric lupus syndrome. Arthritis Rheum 42:599–608
Bombardier C, Gladman DD, Urowitz MB, Caron D, Chang CH (1992) Derivation of the SLEDAI. A disease activity index for lupus patients. The Committee on Prognosis Studies in SLE. Arthritis Rheum 35:630–640
Gladman DD, Urowitz MB, Goldsmith CH, Fortin P, Ginzler E, Gordon C, Hanly JG et al (1997) The reliability of the systemic lupus international collaborating clinics/American college of rheumatology damage index in patients with systemic lupus erythematosus. Arthritis Rheum 40:809–813
Brunner HI, Silverman ED, To T, Bombardier C, Feldman BM (2002) Factors for damage in childhood-onset systemic lupus erythematosus: cumulative disease activity and medication use predict disease damage. Arthritis Rheum 46:436–444
Brandt JT, Triplett DA, Scharer I (1995) Criteria for the diagnosis of lupus anticoagulant: an update. Thromb Haemost 74:1185–1190
Singsen BH (1990) Epidemiology of rheumatic diseases: rheumatic diseases in childhood. Rheum Dis Clin North Am 16:581–599
Dubois EL, Tuffanelli DL (1964) Clinical manifestations of systemic lupus erythematosus. JAMA 190:104–113
Pande I, Sekharan NG, Kailash S, Uppal SS, Singh RR, Kumar A, Malaviya AN (1993) Analysis of clinical and laboratory profile in Indian childhood systemic lupus erythematosus and its comparison with SLE in adults. Lupus 2:83–87
Glidden RS, Mantzouranis EC, Borel Y (1983) Systemic lupus erythematosus in childhood: clinical manifestations and improved survival in 55 patients. Clin Immunol Immunopathol 29:196–210
Lehman TJ, McCurdy DK, Bernstein BH, King KK, Hanson V (1989) Systemic lupus erythematosus in the first decade of life. Pediatrics 83:235–239
Marini R, Costallat LT (1999) Young age at onset, renal involvement, and arterial hypertension are of adverse prognostic significance in juvenile systemic lupus erythematosus. Rev Rhum Engl Ed 66:303–309
Gloor JM (1998) Lupus nephritis in children. Lupus 7:639–643
Rood MJ, Ten Cate R, van Suijlekom-Smit LW, den Ouden EJ, Ouwerkerk FE, Breedveld FC, Huizinga TW (1999) Childhood-onset systemic lupus erythematosus: clinical presentation and prognosis in 31 patients. Scand J Rheumatol 28:222–226
Emre S, Bilge I, Sirin A, Kilicaslan I, Nayir A, Oktem F, Uysal V (2001) Lupus nephritis in children: prognostic significance of clinicopathological findings. Nephron 87:118–126
Lehman TJ (2001) Modern treatment of childhood SLE. Clin Exp Rheumatol 19:487–489
Gedalia A, Molina JF, Molina J, Uribe O, Malagon C, Espinoza LR (1999) Childhood-onset systemic lupus erythematosus: a comparative study of African Americans and Latin Americans. J Natl Med Assoc 91:497–501
Hagelberg S, Lee Y, Bargman J, Mah G, Schneider R, Laskin C, Eddy A et al (2002) Longterm followup of childhood lupus nephritis. J Rheumatol 29:2635–2642
McCurdy DK, Lehman TJ, Bernstein B, Hanson V, King KK, Nadorra R, Landing BH (1992) Lupus nephritis: prognostic factors in children. Pediatrics 89:240–246
Haji Muhammad Ismail Hussain I, Loh WF, Sofiah A (1999) Childhood cerebral lupus in an Oriental population. Brain Dev 21:229–235
Brunner HI, Feldman BM, Bombardier C, Silverman ED (1999) 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 Rheum 4:1354–1360
Schor NF (2000) Neurology of systemic autoimmune disorders: a pediatric perspective. Semin Pediatr Neurol 7:108–117
Chan DQ (2000) Neurologic, ophthalmic, and neuropsychiatric manifestations of pediatric systemic lupus erythematosus. Optom Vis Sci 77:388–394
Lee T, von Scheven E, Sandborg C (2001) Systemic lupus erythematosus and antiphospholipid syndrome in children and adolescents. Curr Opin Rheumatol 13:415–421
Ward MM, Studenski S (1990) Systemic lupus erythematosus in men: a multivariate analysis of gender differences in clinical manifestations. J Rheumatol 17:220–224
Mok CC, Lau CS, Chan TM, Wong RW (1999) Clinical characteristics and outcome of southern Chinese males with systemic lupus erythematosus. Lupus 8:188–196
Costallat LTL, Coimbra AM (1993) Systemic lupus erythematosus in 18 Brazilian males: clinical and laboratory analysis. Clin Rheumatol 12:522–525
Voulgari PV, Katsimbri P, Alamanos Y, Drosos AA (2002) Gender and age differences in systemic lupus erythematosus. A study of 489 Greek patients with a review of the literature. Lupus 11:722–729
Mok CC, Lau CS, Chan TM, Wong RW (1999) Clinical characteristics and outcome of southern Chinese males with systemic lupus erythematosus. Lupus 8:188–196
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The work was supported by Fundos Remanescentes da Sociedade Brasileira de Reumatologia
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Appenzeller, S., Marini, R. & Costallat, L.T. Damage did not independently influence mortality in childhood systemic lupus erythematosus. Rheumatol Int 25, 619–624 (2005). https://doi.org/10.1007/s00296-004-0552-z
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DOI: https://doi.org/10.1007/s00296-004-0552-z