Introduction: The aim of this study was to assess the differences between childhood-onset and adult-onset systemic lupus erythematosus (cSLE and aSLE) for clinical manifestations and mortality using a meta-analytic approach. Methods: The PubMed, EMBASE, and the Cochrane library were searched for eligible studies published between January 1982 and March 2021. The odds ratio (OR) with 95% confidence interval was used to calculate the pooled effect estimates using the random-effects model. Results: Thirty-four studies involving 21,946 SLE patients were included. cSLE was associated with an increased risk of malar rash (OR: 1.64; p < 0.001), ulcers/mucocutaneous involvement (OR: 1.22; p = 0.039), general neurological involvement (OR: 1.52; p < 0.001), seizures (OR: 1.92; p < 0.001), general renal involvement (OR: 2.08; p < 0.001), proteinuria (OR: 1.35; p = 0.015), urinary cellular casts (OR: 1.67; p = 0.047), fever (OR: 2.31; p < 0.001), anemia (OR: 1.91; p < 0.001), thrombocytopenia (OR: 1.41; p < 0.001), leucopenia (OR: 1.57; p = 0.017), lymphadenopathy (OR: 2.40; p < 0.001), and cutaneous vasculitis (OR: 1.72; p = 0.001) as compared with aSLE. Moreover, cSLE versus aSLE was associated with a reduced risk of articular manifestations (OR: 0.63; p = 0.001), pulmonary involvement (OR: 0.54; p = 0.001), and pleuritis (OR: 0.61; p < 0.001). There were no significant differences between cSLE and aSLE for mortality risk (OR: 1.20; p = 0.203). Conclusion: We found that certain clinical manifestations of SLE are different in cSLE and aSLE. Moreover, the mortality risk of cSLE and aSLE was not significantly different.

Jorge AM, Lu N, Zhang Y, Rai SK, Choi HK. Unchanging premature mortality trends in systemic lupus erythematosus: a general population-based study (1999–2014).
. 2018 Feb 1;57(2):337–44.
Dörner T, Furie R. Novel paradigms in systemic lupus erythematosus.
. 2019 Jun 8;393(10188):2344–58.
Durcan L, O’Dwyer T, Petri M. Management strategies and future directions for systemic lupus erythematosus in adults.
. 2019 Jun 8;393(10188):2332–43.
Felten R, Sagez F, Gavand PE, Martin T, Korganow AS, Sordet C, et al. 10 most important contemporary challenges in the management of SLE.
Lupus Sci Med
. 2019;6(1):e000303.
Tsokos GC. Systemic lupus erythematosus.
N Engl J Med
. 2011 Dec 1;365(22):2110–21.
Hedrich CM, Smith EMD, Beresford MW. Juvenile-onset systemic lupus erythematosus (jSLE): pathophysiological concepts and treatment options.
Best Pract Res Clin Rheumatol
. 2017 Aug;31(4):488–504.
Ambrose N, Morgan TA, Galloway J, Ionnoau Y, Beresford MW, Isenberg DA. Differences in disease phenotype and severity in SLE across age groups.
. 2016 Dec;25(14):1542–50.
Moher D, Liberati A, Tetzlaff J, Altman DG. Preferred reporting items for systematic reviews and meta-analyses: the PRISMA statement.
. 2009 Jul 21;339(7):b2535.
Wells GA, Shea B, O’Connell D, Peterson J, Welch V, Losos M, et al.
The Newcastle-Ottawa Scale (NOS) for assessing the quality of nonrandomised studies in meta-analyses
. Ottawa (ON): Ottawa Hospital Research Institute; 2009.
Ades AE, Lu G, Higgins JP. The interpretation of random-effects meta-analysis in decision models.
Med Decis Making
. 2005 Nov–Dec;25(6):646–54.
DerSimonian R, Laird N. Meta-analysis in clinical trials revisited.
Contemp Clin Trials
. 2015 Nov;45(Pt A):139–45.
Higgins JP, Thompson SG, Deeks JJ, Altman DG. Measuring inconsistency in meta-analyses.
. 2003 Sep 6;327(7414):557–60.
Deeks JJ, Higgins JP, Altman DG. Analysing data and undertaking meta-analyses. In: Higgins J, Green S, editors.
Cochrane handbook for systematic reviews of interventions
. Oxford: The Cochrane Collaboration; 2008. p. 243–96.
Begg CB, Mazumdar M. Operating characteristics of a rank correlation test for publication bias.
. 1994 Dec;50(4):1088–101.
Egger M, Davey Smith G, Schneider M, Minder C. Bias in meta-analysis detected by a simple, graphical test.
. 1997 Sep 13;315(7109):629–34.
Hashimoto H, Tsuda H, Hirano T, Takasaki Y, Matsumoto T, Hirose S. Differences in clinical and immunological findings of systemic lupus erythematosus related to age.
J Rheumatol
. 1987 Jun;14(3):497–501.
Cervera R, Khamashta MA, Font J, Sebastiani GD, Gil A, Lavilla P, et al. 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.
. 1993 Mar;72(2):113–24.
Pande I, Sekharan NG, Kailash S, Uppal SS, Singh RR, Kumar A, et al. Analysis of clinical and laboratory profile in Indian childhood systemic lupus erythematosus and its comparison with SLE in adults.
. 1993 Apr;2(2):83–7.
Vlachoyiannopoulos PG, Karassa FB, Karakostas KX, Drosos AA, Moutsopoulos HM. Systemic lupus erythematosus in Greece. Clinical features, evolution and outcome: a descriptive analysis of 292 patients.
. 1993 Oct;2(5):303–12.
Costallat LT, Coimbra AM. Systemic lupus erythematosus: clinical and laboratory aspects related to age at disease onset.
Clin Exp Rheumatol
. 1994 Nov–Dec;12(6):603–7.
Janwityanujit S, Totemchokchyakarn K, Verasertniyom O, Vanichapuntu M, Vatanasuk M. Age-related differences on clinical and immunological manifestations of SLE.
Asian Pac J Allergy Immunol
. 1995 Dec;13(2):145–9.
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 Rheumatol
. 1995 Sep;34(9):866–72.
Font J, Cervera R, Espinosa G, Pallarés L, Ramos-Casals M, Jiménez S, et al. 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
. 1998 Aug;57(8):456–9.
Carreño L, López-Longo FJ, Monteagudo I, Rodríguez-Mahou M, Bascones M, González CM, et al. Immunological and clinical differences between juvenile and adult onset of systemic lupus erythematosus.
. 1999;8(4):287–92.
Rood MJ, ten Cate R, van Suijlekom-Smit LW, den Ouden EJ, Ouwerkerk FE, Breedveld FC, et al. Childhood-onset Systemic Lupus Erythematosus: clinical presentation and prognosis in 31 patients.
Scand J Rheumatol
. 1999;28(4):222–6.
Mok CC, Mak A, Chu WP, To CH, Wong SN. Long-term survival of southern Chinese patients with systemic lupus erythematosus: a prospective study of all age-groups.
. 2005 Jul;84(4):218–24.
Gómez J, Suárez A, López P, Mozo L, Díaz JB, Gutiérrez C. Systemic lupus erythematosus in Asturias, Spain: clinical and serologic features.
. 2006 May;85(3):157–68.
Brunner HI, Gladman DD, Ibañez D, Urowitz MD, Silverman ED. Difference in disease features between childhood-onset and adult-onset systemic lupus erythematosus.
Arthritis Rheum
. 2008 Feb;58(2):556–62.
Ramírez Gómez LA, Uribe Uribe O, Osio Uribe O, Grisales Romero H, Cardiel MH, Wojdyla D, et al. Childhood systemic lupus erythematosus in Latin America. The GLADEL experience in 230 children.
. 2008 Jun;17(6):596–604.
Tucker LB, Uribe AG, Fernández M, Vilá LM, McGwin G, Apte M, et al. Adolescent onset of lupus results in more aggressive disease and worse outcomes: results of a nested matched case-control study within LUMINA, a multiethnic US cohort (LUMINA LVII).
. 2008 Apr;17(4):314–22.
Hersh AO, von Scheven E, Yazdany J, Panopalis P, Trupin L, Julian L, et al. Differences in long-term disease activity and treatment of adult patients with childhood- and adult-onset systemic lupus erythematosus.
Arthritis Rheum
. 2009 Jan 15;61(1):13–20.
Hoffman IE, Lauwerys BR, De Keyser F, Huizinga TW, Isenberg D, Cebecauer L, et al. Juvenile-onset systemic lupus erythematosus: different clinical and serological pattern than adult-onset systemic lupus erythematosus.
Ann Rheum Dis
. 2009 Mar;68(3):412–5.
Feng JB, Ni JD, Yao X, Pan HF, Li XP, Xu JH, et al. Gender and age influence on clinical and laboratory features in Chinese patients with systemic lupus erythematosus: 1,790 cases.
Rheumatol Int
. 2010 Jun;30(8):1017–23.
Hersh AO, Trupin L, Yazdany J, Panopalis P, Julian L, Katz P, et al. Childhood-onset disease as a predictor of mortality in an adult cohort of patients with systemic lupus erythematosus.
Arthritis Care Res
. 2010 Aug;62(8):1152–9.
Tu YL, Yeh KW, Chen LC, Yao TC, Ou LS, Lee WI, et al. Differences in disease features between childhood-onset and adult-onset systemic lupus erythematosus patients presenting with acute abdominal pain.
Semin Arthritis Rheum
. 2011 Apr;40(5):447–54.
Martínez-Barrio J, Ovalles-Bonilla JG, López-Longo FJ, González CM, Montoro M, Valor L, et al. Juvenile, adult and late-onset systemic lupus erythematosus: a long term follow-up study from a geographic and ethnically homogeneous population.
Clin Exp Rheumatol
. 2015 Nov–Dec;33(6):788–94.
Tarr T, Dérfalvi B, Győri N, Szántó A, Siminszky Z, Malik A, et al. Similarities and differences between pediatric and adult patients with systemic lupus erythematosus.
. 2015 Jul;24(8):796–803.
das Chagas Medeiros MM, Bezerra MC, Braga FN, da Justa Feijão MR, Gois AC, Rebouças VC, et al. 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.
. 2016 Apr;25(4):355–63.
Gormezano NW, Otsuzi CI, Barros DL, da Silva MA, Pereira RM, Campos LM, et al. Macrophage activation syndrome: a severe and frequent manifestation of acute pancreatitis in 362 childhood-onset compared to 1830 adult-onset systemic lupus erythematosus patients.
Semin Arthritis Rheum
. 2016 Jun;45(6):706–10.
Gormezano NW, Silva CA, Aikawa NE, Barros DL, da Silva MA, Otsuzi CI, et al. Chronic arthritis in systemic lupus erythematosus: distinct features in 336 paediatric and 1830 adult patients.
Clin Rheumatol
. 2016 Jan;35(1):227–31.
Sousa S, Gonçalves MJ, Inês LS, Eugénio G, Jesus D, Fernandes S, et al. Clinical features and long-term outcomes of systemic lupus erythematosus: comparative data of childhood, adult and late-onset disease in a national register.
Rheumatol Int
. 2016 Jul;36(7):955–60.
Artim-Esen B, Şahin S, Çene E, Şahinkaya Y, Barut K, Adrovic A, et al. Comparison of disease characteristics, organ damage, and survival in patients with juvenile-onset and adult-onset systemic lupus erythematosus in a combined cohort from 2 tertiary centers in Turkey.
J Rheumatol
. 2017 May;44(5):619–25.
Fatemi A, Matinfar M, Smiley A. Childhood versus adult-onset systemic lupus erythematosus: long-term outcome and predictors of mortality.
Clin Rheumatol
. 2017 Feb;36(2):343–50.
Gormezano NW, Kern D, Pereira OL, Esteves GC, Sallum AM, Aikawa NE, et al. Autoimmune hemolytic anemia in systemic lupus erythematosus at diagnosis: differences between pediatric and adult patients.
. 2017 Apr;26(4):426–30.
Sassi RH, Hendler JV, Piccoli GF, Gasparin AA, da Silva Chakr RM, Brenol JC, et al. Age of onset influences on clinical and laboratory profile of patients with systemic lupus erythematosus.
Clin Rheumatol
. 2017 Jan;36(1):89–95.
Chang JC, Xiao R, Mercer-Rosa L, Knight AM, Weiss PF. Child-onset systemic lupus erythematosus is associated with a higher incidence of myopericardial manifestations compared to adult-onset disease.
. 2018 Nov;27(13):2146–54.
El Hadidi KT, Medhat BM, Abdel Baki NM, Abdel Kafy H, Abdelrahaman W, Yousri AY, et al. Characteristics of systemic lupus erythematosus in a sample of the Egyptian population: a retrospective cohort of 1109 patients from a single center.
. 2018 May;27(6):1030–8.
Esteves GCX, Gormezano NWS, Pereira OL, Kern D, Silva CA, Pereira RMR, et al. Distinct clinical correlates of immune thrombocytopenic purpura at diagnosis of childhood-onset and adult SLE.
Mod Rheumatol
. 2018 Jul;28(4):649–53.
Fonseca R, Aguiar F, Rodrigues M, Brito I. Clinical phenotype and outcome in lupus according to age: a comparison between juvenile and adult onset.
Reumatol Clin
. 2018 May–Jun;14(3):160–3.
Livingston B, Bonner A, Pope J. Differences in clinical manifestations between childhood-onset lupus and adult-onset lupus: a meta-analysis.
. 2011 Nov;20(13):1345–55.
Bundhun PK, Kumari A, Huang F. Differences in clinical features observed between childhood-onset versus adult-onset systemic lupus erythematosus: a systematic review and meta-analysis.
. 2017 Sep;96(37):e8086.
Webb R, Kelly JA, Somers EC, Hughes T, Kaufman KM, Sanchez E, et al. 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
. 2011 Jan;70(1):151–6.
Brunner HI, Silverman ED, To T, Bombardier C, Feldman BM. Risk factors for damage in childhood-onset systemic lupus erythematosus: cumulative disease activity and medication use predict disease damage.
Arthritis Rheum
. 2002 Feb;46(2):436–44.
Petri M, Purvey S, Fang H, Magder LS. Predictors of organ damage in systemic lupus erythematosus: the Hopkins Lupus Cohort.
Arthritis Rheum
. 2012 Dec;64(12):4021–8.
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.