Abstract
The present study aims to evaluate differences in clinical and laboratory manifestations and medication use in the different ages of disease onset in patients with systemic lupus erythematosus (SLE). This cross-sectional study consisted of 598 SLE patients (550 female and 48 male), who attended the Rheumatology Clinic of the Hospital de Clínicas de Porto Alegre between 2003 and 2015. Demographic, clinical and laboratory data were collected. The patients were classified into three groups according to their ages at disease diagnosis. Mean age of diagnosis was 33.6 ± 14.3 years, and the median (25th–75th percentile) disease duration was 13 (7–20) years. Among the patients studied, 419 (70%) were adult-onset (aSLE), 90 (14.8%) were late-onset (lSLE) and 89 (14.8%) were childhood-onset (cSLE). The female to male ratio was higher in aSLE (18:1) compared to the other groups (p = 0.001). Arthritis was predominantly found in aSLE (78.5%) when compared with lSLE (57.7%) (p < 0.001). Nephritis was more common in cSLE (60.6%) than in lSLE (26.6%) (p < 0.001). Median (25th–75th percentile) of SLE disease activity index (SLEDAI) was higher in the cSLE group [2 (0–5)] when compared to the lSLE group [0 (0–4)] (p = 0.045). Childhood-onset SLE showed a more severe disease due to the higher incidence of nephritis and needed a more aggressive treatment with immunosuppressive drugs.
Similar content being viewed by others
References
Monticielo OA, Mucenic T, Xavier RM et al (2008) The role of mannose-binding lectin in systemic lupus erythematosus. Clin Rheumatol 27(4):413–419
Boddaert J, Huong DL, Amoura Z et al (2004) Late-onset systemic lupus erythematosus: a personal series of 47 patients and pooled analysis of 714 cases in the literature. Medicine (Baltimore) 83(6):348–359
Ballou SP, Khan MA, Kushner I (1982) Clinical features of systemic lupus erythematosus: differences related to race and age of onset. Arthritis Rheum 25(1):55–60
Alonso MD, Martinez-Vazquez F, de Teran TD et al (2012) Late-onset systemic lupus erythematosus in northwestern Spain: differences with early-onset systemic lupus erythematosus and literature review. Lupus 21(10):1135–1148
Bertoli AM, Alarcon GS, Calvo-Alen J et al (2006) Systemic lupus erythematosus in a multiethnic US cohort. XXXIII. Clinical [corrected] features, course, and outcome in patients with late-onset disease. Arthritis Rheum 54(5):1580–1587
Ho CT, Mok CC, Lau CS et al (1998) Late onset systemic lupus erythematosus in southern Chinese. Ann Rheum Dis 57(7):437–440
Chen YM, Lin CH, Chen HH et al (2014) Onset age affects mortality and renal outcome of female systemic lupus erythematosus patients: a nationwide population-based study in Taiwan. Rheumatology (Oxford) 53(1):180–185
Feng X, Zou Y, Pan W et al (2014) Associations of clinical features and prognosis with age at disease onset in patients with systemic lupus erythematosus. Lupus 23(3):327–334
Watson L, Leone V, Pilkington C et al (2012) Disease activity, severity, and damage in the UK juvenile-onset systemic lupus erythematosus cohort. Arthritis Rheum 64(7):2356–2365
Brunner HI, Gladman DD, Ibanez D et al (2008) Difference in disease features between childhood-onset and adult-onset systemic lupus erythematosus. Arthritis Rheum 58(2):556–562
Lazaro D (2007) Elderly-onset systemic lupus erythematosus: prevalence, clinical course and treatment. Drugs Aging 24(9):701–715
Rovensky J, Tuchynova A (2008) Systemic lupus erythematosus in the elderly. Autoimmun Rev 7(3):235–239
das Chagas Medeiros MM, Bezerra MC, Braga FN et al (2016) Clinical and immunological aspects and outcome of a Brazilian cohort of 414 patients with systemic lupus erythematosus (SLE): comparison between childhood-onset, adult-onset, and late-onset SLE. Lupus 25(4):355–363
Costallat LT, Coimbra AM (1994) Systemic lupus erythematosus: clinical and laboratory aspects related to age at disease onset. Clin Exp Rheumatol 12(6):603–607
Sato VA, Marques ID, Goldenstein PT, et al. (2012) Lupus nephritis is more severe in children and adolescents than in older adults. 21(9):978–983
Borba EF, Araujo DB, Bonfa E et al (2013) Clinical and immunological features of 888 Brazilian systemic lupus patients from a monocentric cohort: comparison with other populations. Lupus 22(7):744–749
Chahade WH, Sato EI, Moura JE Jr et al (1995) Systemic lupus erythematosus in Sao Paulo/Brazil: a clinical and laboratory overview. Lupus 4(2):100–103
Nakashima CA, Galhardo AP, Silva JF et al (2011) Incidence and clinical-laboratory aspects of systemic lupus erythematosus in a southern Brazilian city. Rev Bras Reumatol 51(3):231–239
Vilar MJ, Sato EI (2002) Estimating the incidence of systemic lupus erythematosus in a tropical region (Natal, Brazil). Lupus 11(8):528–532
Tan EM, Cohen AS, Fries JF et al (1982) The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 25(11):1271–1277
Hochberg MC (1997) Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 40(9):1725
Monticielo OA, Brenol JC, Chies JA et al (2012) The role of BsmI and FokI vitamin D receptor gene polymorphisms and serum 25-hydroxyvitamin D in Brazilian patients with systemic lupus erythematosus. Lupus 21(1):43–52
Santos NPC, Ribeiro-Rodrigues EM, Ribeiro-dos-Santos Andrea KC et al (2010) Assessing individual interethnic admixture and population substructure using a 48-insertion-deletion (INSEL) ancestry-informative marker (AIM) panel. Hum Mutat 31(2):184–190
Cervera R, Khamashta MA, Font J et al (1993) Systemic lupus erythematosus: clinical and immunologic patterns of disease expression in a cohort of 1,000 patients. The European Working Party on Systemic Lupus Erythematosus. Medicine (Baltimore) 72(2):113–124
Feng JB, Ni JD, Yao X et al (2010) Gender and age influence on clinical and laboratory features in Chinese patients with systemic lupus erythematosus: 1,790 cases. Rheumatol Int 30(8):1017–1023
Silva CA, Avcin T, Brunner HI (2012) Taxonomy for systemic lupus erythematosus with onset before adulthood. Arthritis Care Res (Hoboken) 64(12):1787–1793
Wilson WA, Gharavi AE, Koike T et al (1999) International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome: report of an international workshop. Arthritis Rheum 42(7):1309–1311
Vitali C, Bombardieri S, Jonsson R et al (2002) Classification criteria for Sjogren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 61(6):554–558
Vitali C (2003) Classification criteria for Sjogren’s syndrome. Ann Rheum Dis 62(1):94–95
Miyakis S, Lockshin MD, Atsumi T et al (2006) International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost 4(2):295–306
Bombardier C, Gladman DD, Urowitz MB et al (1992) Derivation of the SLEDAI. A disease activity index for lupus patients. The Committee on Prognosis Studies in SLE. Arthritis Rheum 35(6):630–640
Gladman D, Ginzler E, Goldsmith C et al (1996) The development and initial validation of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index for systemic lupus erythematosus. Arthritis Rheum 39(3):363–369
Romero-Diaz J, Isenberg D, Ramsey-Goldman R (2011) Measures of adult systemic lupus erythematosus: updated version of British Isles Lupus Assessment Group (BILAG 2004), European Consensus Lupus Activity Measurements (ECLAM), Systemic Lupus Activity Measure, Revised (SLAM-R), Systemic Lupus Activity Questionnaire for Population Studies (SLAQ), Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K), and Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI). Arthritis Care Res (Hoboken) 63 Suppl 11:S37–S46
Griffiths B, Mosca M, Gordon C (2005) Assessment of patients with systemic lupus erythematosus and the use of lupus disease activity indices. Best Pract Res Clin Rheumatol 19(5):685–708
Font J, Cervera R, Navarro M et al (1992) Systemic lupus erythematosus in men: clinical and immunological characteristics. Ann Rheum Dis 51(9):1050–1052
Formiga F, Moga I, Pac M et al (1999) Mild presentation of systemic lupus erythematosus in elderly patients assessed by SLEDAI. SLE Disease Activity Index. Lupus 8(6):462–465
Lalani S, Pope J, de Leon F et al (2010) Clinical features and prognosis of late-onset systemic lupus erythematosus: results from the 1000 faces of lupus study. J Rheumatol 37(1):38–44
Webb R, Kelly JA, Somers EC et al (2011) Early disease onset is predicted by a higher genetic risk for lupus and is associated with a more severe phenotype in lupus patients. Ann Rheum Dis 70(1):151–156
Catoggio LJ, Soriano ER, Imamura PM et al (2015) Late-onset systemic lupus erythematosus in Latin Americans: a distinct subgroup? Lupus 24(8):788–795
Achour A, Mankai A, Thabet Y et al (2012) Systemic lupus erythematosus in the elderly. Rheumatol Int 32(5):1225–1229
Koh ET, Boey ML (1994) Late onset lupus: a clinical and immunological study in a predominantly Chinese population. J Rheumatol 21(8):1463–1467
Padovan M, Govoni M, Castellino G et al (2007) Late onset systemic lupus erythematosus: no substantial differences using different cut-off ages. Rheumatol Int 27(8):735–741
McMurray RW, May W (2003) Sex hormones and systemic lupus erythematosus: review and meta-analysis. Arthritis Rheum 48(8):2100–2110
Cartella S, Cavazzana I, Ceribelli A et al (2013) Evaluation of mortality, disease activity, treatment, clinical and immunological features of adult and late onset systemic lupus erythematosus. Autoimmunity 46(6):363–368
Costenbader KH, Feskanich D, Stampfer MJ et al (2007) Reproductive and menopausal factors and risk of systemic lupus erythematosus in women. Arthritis Rheum 56(4):1251–1262
Livingston B, Bonner A, Pope J (2011) Differences in clinical manifestations between childhood-onset lupus and adult-onset lupus: a meta-analysis. Lupus 20(13):1345–1355
Gómez LAR, Uribe OU, Uribe OO et al (2008) Childhood systemic lupus erythematosus in Latin America. The GLADEL experience in 230 children. Lupus 17(6):596–604
Domenech I, Aydintug O, Cervera R et al (1992) Systemic lupus erythematosus in 50 year olds. Postgrad Med J 68(800):440–444
Hochberg MC, Boyd RE, Ahearn JM et al (1985) Systemic lupus erythematosus: a review of clinico-laboratory features and immunogenetic markers in 150 patients with emphasis on demographic subsets. Medicine (Baltimore) 64(5):285–295
Jonsson H, Nived O, Sturfelt G (1988) The effect of age on clinical and serological manifestations in unselected patients with systemic lupus erythematosus. J Rheumatol 15(3):505–509
Franceschini F, Cavazzana I (2005) Anti-Ro/SSA and La/SSB antibodies. Autoimmunity 38(1):55–63
Catoggio LJ, Skinner RP, Smith G et al (1984) Systemic lupus erythematosus in the elderly: clinical and serological characteristics. J Rheumatol 11(2):175–181
Mak SK, Lam EK, Wong AK (1998) Clinical profile of patients with late-onset SLE: not a benign subgroup. Lupus 7(1):23–28
Hashimoto H, Tsuda H, Hirano T et al (1987) Differences in clinical and immunological findings of systemic lupus erythematosus related to age. J Rheumatol 14(3):497–501
Descloux E, Durieu I, Cochat P et al (2009) Influence of age at disease onset in the outcome of paediatric systemic lupus erythematosus. Rheumatology (Oxford) 48(7):779–784
Malattia C, Martini A (2013) Paediatric-onset systemic lupus erythematosus. Best Pract Res Clin Rheumatol 27(3):351–362
Livingston B, Bonner A, Pope J (2012) Differences in autoantibody profiles and disease activity and damage scores between childhood- and adult-onset systemic lupus erythematosus: a meta-analysis. Semin Arthritis Rheum 42(3):271–280
Marks SD, Tullus K (2010) Modern therapeutic strategies for paediatric systemic lupus erythematosus and lupus nephritis. Acta Paediatr 99(7):967–974
Ruiz-Irastorza G, Danza A, Khamashta M (2013) Treatment of systemic lupus erythematosus: myths, certainties and doubts. Med Clin (Barc) 141(12):533–542
Acknowledgements
Fundo de Incentivo à Pesquisa e Eventos-Research Support Fund of the Clinical Hospital of Porto Alegre.
Coordenação de Aperfeiçoamento de Pessoal de Nível Superior.
Ministério da Ciência, Tecnologia e Inovação-Conselho Nacional de Desenvolvimento Científico e Tecnológico.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Disclosures
None.
Rights and permissions
About this article
Cite this article
Sassi, R.H., Hendler, J.V., Piccoli, G.F. et al. Age of onset influences on clinical and laboratory profile of patients with systemic lupus erythematosus. Clin Rheumatol 36, 89–95 (2017). https://doi.org/10.1007/s10067-016-3478-4
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10067-016-3478-4