Skip to main content

Advertisement

SpringerLink
Log in

Clinical and laboratory features, disease activity, and outcomes of juvenile systemic lupus erythematosus at diagnosis: a single-center study from southern China

  • Original Article
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

Abstract

Objectives

This retrospective study aimed to analyze the clinical and laboratory features, disease activity, and outcomes of juvenile systemic lupus erythematosus (jSLE) patients from southern China.

Methods

A total of 173 jSLE patients who attended Rheumatology and Immunology Department of Meizhou People’s Hospital between January 2010 and December 2019 are included for analysis.

Results

The mean age of disease onset was 13.65 ± 2.80 (range, 5–17). The median age at diagnosis was 13.98 ± 2.88 (range, 5–17) years. The female to male ratio was 6.5:1. Renal involvement was the most prevalent clinical feature, occurred in 71.7% of the patients. A total of 27 (15.6%) patients underwent renal biopsy, and the most common type of renal pathology was class IV (44.4%). Our study demonstrated differences in antibody clusters for which the positivity rates of anti-dsDNA antibodies and anti-Sm antibodies were higher than the other jSLE cohorts reported in China and worldwide. Cyclophosphamide combined with corticosteroids was the main treatment medication. The mean SLE Disease Activity Index (SLEDAI) score at diagnosis was 14.3 ± 7.6 (range, 3–38). Five patients died during the initial diagnosis and treatment. Infection was the major cause of death.

Conclusion

The jSLE patients in this cohort had a higher prevalence of renal involvement, anti-dsDNA antibodies, and anti-Sm antibodies. Multicenter studies are needed to clarify the different clinical features of jSLE in southern China.

Key Points

• A single-center study of juvenile systemic lupus erythematosus in clinical and laboratory features.

• The jSLE patients had a higher prevalence of renal involvement and antibody disorder.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Rahman A, Isenberg DA (2008) Systemic lupus erythematosus. N Engl J Med 358(9):929–939. https://doi.org/10.1056/NEJMra071297

    Article  CAS  PubMed  Google Scholar 

  2. Pineles D, Valente A, Warren B, Peterson MG, Lehman TJ, Moorthy LN (2011) Worldwide incidence and prevalence of pediatric onset systemic lupus erythematosus. Lupus 20(11):1187–1192. https://doi.org/10.1177/0961203311412096

    Article  CAS  PubMed  Google Scholar 

  3. Lewis MJ, Jawad AS (2017) The effect of ethnicity and genetic ancestry on the epidemiology, clinical features and outcome of systemic lupus erythematosus. Rheumatology (Oxford) 56(suppl_1):i67–i77. https://doi.org/10.1093/rheumatology/kew399

    Article  CAS  Google Scholar 

  4. Sassi RH, Hendler JV, Piccoli GF, Gasparin AA, da Silva Chakr RM, Brenol JC et al (2017) Age of onset influences on clinical and laboratory profile of patients with systemic lupus erythematosus. Clin Rheumatol 36(1):89–95. https://doi.org/10.1007/s10067-016-3478-4

    Article  PubMed  Google Scholar 

  5. Fatemi A, Matinfar M, Smiley A (2017) Childhood versus adult-onset systemic lupus erythematosus: long-term outcome and predictors of mortality. Clin Rheumatol 36(2):343–350. https://doi.org/10.1007/s10067-016-3509-1

    Article  PubMed  Google Scholar 

  6. Hersh A, von Scheven E, Yelin E (2011) Adult outcomes of childhood-onset rheumatic diseases. Nat Rev Rheumatol 7(5):290–295. https://doi.org/10.1038/nrrheum.2011.38

    Article  PubMed  PubMed Central  Google Scholar 

  7. Feng X, Zou Y, Pan W, Wang X, Wu M, Zhang M 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. https://doi.org/10.1177/0961203313513508

    Article  CAS  PubMed  Google Scholar 

  8. Wu CY, Li CF, Wu QJ, Xu JH, Jiang LD, Gong L et al (2017) Chinese systemic lupus erythematosus treatment and research group registry IX: clinical features and survival of childhood-onset systemic lupus erythematosus in China. Chin Med J (Engl) 130(11):1276–1282. https://doi.org/10.4103/0366-6999.206346

    Article  Google Scholar 

  9. Artim-Esen B, Sahin S, Cene E, Sahinkaya Y, Barut K, Adrovic A et al (2017) 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 44(5):619–625. https://doi.org/10.3899/jrheum.160340

    Article  PubMed  Google Scholar 

  10. Choi JH, Park DJ, Kang JH, Yim YR, Lee KE, Lee JW et al (2015) Comparison of clinical and serological differences among juvenile-, adult-, and late-onset systemic lupus erythematosus in Korean patients. Lupus 24(12):1342–1349. https://doi.org/10.1177/0961203315591024

    Article  CAS  PubMed  Google Scholar 

  11. Bundhun PK, Kumari A, Huang F (2017) Differences in clinical features observed between childhood-onset versus adult-onset systemic lupus erythematosus: a systematic review and meta-analysis. Medicine (Baltimore) 96(37):e8086. https://doi.org/10.1097/MD.0000000000008086

    Article  Google Scholar 

  12. Hersh AO, von Scheven E, Yazdany J, Panopalis P, Trupin L, Julian L et al (2009) Differences in long-term disease activity and treatment of adult patients with childhood- and adult-onset systemic lupus erythematosus. Arthritis Rheum 61(1):13–20. https://doi.org/10.1002/art.24091

    Article  PubMed  PubMed Central  Google Scholar 

  13. Hochberg MC (1997) Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 40(9):1725. https://doi.org/10.1002/art.1780400928

    Article  CAS  PubMed  Google Scholar 

  14. The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes (1999). Arthritis Rheum 42 (4):599–608. https://doi.org/10.1002/1529-0131(199904)42:43.0.CO;2-F

  15. Weening JJ, D’Agati VD, Schwartz MM, Seshan SV, Alpers CE, Appel GB et al (2004) The classification of glomerulonephritis in systemic lupus erythematosus revisited. Kidney Int 65(2):521–530. https://doi.org/10.1111/j.1523-1755.2004.00443.x

    Article  PubMed  Google Scholar 

  16. Gladman DD, Ibanez D, Urowitz MB (2002) Systemic lupus erythematosus disease activity index 2000. J Rheumatol 29(2):288–291

    PubMed  Google Scholar 

  17. Setoue DN, Pitta AC, Fiorot FJ, Nastri MM, Novak GV, Molinari BC et al (2018) Symptomatic polyautoimmunity at diagnosis of 1463 childhood-onset lupus: a Brazilian multicenter study. Autoimmun Rev 17(8):836–839. https://doi.org/10.1016/j.autrev.2018.03.009

    Article  PubMed  Google Scholar 

  18. Kim H, Levy DM, Silverman ED, Hitchon C, Bernatsky S, Pineau C et al (2019) A comparison between childhood and adult onset systemic lupus erythematosus adjusted for ethnicity from the 1000 Canadian Faces of Lupus Cohort. Rheumatology (Oxford). https://doi.org/10.1093/rheumatology/kez006

    Article  PubMed Central  Google Scholar 

  19. Tan JH, Hoh SF, Win MT, Chan YH, Das L, Arkachaisri T (2015) Childhood-onset systemic lupus erythematosus in Singapore: clinical phenotypes, disease activity, damage, and autoantibody profiles. Lupus 24(9):998–1005. https://doi.org/10.1177/0961203315584413

    Article  CAS  PubMed  Google Scholar 

  20. Aggarwal A, Phatak S, Srivastava P, Lawrence A, Agarwal V, Misra R (2018) Outcomes in juvenile onset lupus: single center cohort from a developing country. Lupus 27(11):1867–1875. https://doi.org/10.1177/0961203318791046

    Article  CAS  PubMed  Google Scholar 

  21. Gulay CB, Dans LF (2011) Clinical presentations and outcomes of Filipino juvenile systemic lupus erythematosus. Pediatr Rheumatol Online J 9:7. https://doi.org/10.1186/1546-0096-9-7

    Article  PubMed  PubMed Central  Google Scholar 

  22. Ramirez Gomez LA, Uribe Uribe O, Osio Uribe O, Grisales Romero H, Cardiel MH, Wojdyla D et al (2008) Childhood systemic lupus erythematosus in Latin America. The GLADEL experience in 230 children. Lupus 17(6):596–604. https://doi.org/10.1177/0961203307088006

    Article  CAS  PubMed  Google Scholar 

  23. Sahin Y, Sahin S, Adrovic A, Kutlu T, Cokugras FC, Barut K et al (2019) Serological screening for celiac disease in children with systemic lupus erythematosus. Eur J Rheumatol 6(3):142–145. https://doi.org/10.5152/eurjrheum.2019.18130

    Article  PubMed  PubMed Central  Google Scholar 

  24. Lim LSH, Pullenayegum E, Feldman BM, Lim L, Gladman DD, Silverman ED (2018) From childhood to adulthood: disease activity trajectories in childhood-onset systemic lupus erythematosus. Arthritis Care Res (Hoboken) 70(5):750–757. https://doi.org/10.1002/acr.23319

    Article  Google Scholar 

  25. Hiraki LT, Benseler SM, Tyrrell PN, Hebert D, Harvey E, Silverman ED (2008) Clinical and laboratory characteristics and long-term outcome of pediatric systemic lupus erythematosus: a longitudinal study. J Pediatr 152(4):550–556. https://doi.org/10.1016/j.jpeds.2007.09.019

    Article  PubMed  Google Scholar 

  26. Tektonidou MG, Lewandowski LB, Hu J, Dasgupta A, Ward MM (2017) Survival in adults and children with systemic lupus erythematosus: a systematic review and Bayesian meta-analysis of studies from 1950 to 2016. Ann Rheum Dis 76(12):2009–2016. https://doi.org/10.1136/annrheumdis-2017-211663

    Article  PubMed  Google Scholar 

  27. Hiraki LT, Benseler SM, Tyrrell PN, Harvey E, Hebert D, Silverman ED (2009) Ethnic differences in pediatric systemic lupus erythematosus. J Rheumatol 36(11):2539–2546. https://doi.org/10.3899/jrheum.081141

    Article  PubMed  Google Scholar 

  28. Couture J, Silverman ED (2016) Update on the pathogenesis and treatment of childhood-onset systemic lupus erythematosus. Curr Opin Rheumatol 28(5):488–496. https://doi.org/10.1097/BOR.0000000000000317

    Article  CAS  PubMed  Google Scholar 

  29. Brunner HI, Higgins GC, Wiers K, Lapidus SK, Olson JC, Onel K et al (2009) Health-related quality of life and its relationship to patient disease course in childhood-onset systemic lupus erythematosus. J Rheumatol 36(7):1536–1545. https://doi.org/10.3899/jrheum.081164

    Article  PubMed  Google Scholar 

  30. Cavalcante EG, Aikawa NE, Lozano RG, Lotito AP, Jesus AA, Silva CA (2011) Chronic polyarthritis as the first manifestation of juvenile systemic lupus erythematosus patients. Lupus 20(9):960–964. https://doi.org/10.1177/0961203311400113

    Article  CAS  PubMed  Google Scholar 

  31. Joo YB, Park SY, Won S, Bae SC (2016) Differences in clinical features and mortality between childhood-onset and adult-onset systemic lupus erythematosus: a prospective single-center study. J Rheumatol 43(8):1490–1497. https://doi.org/10.3899/jrheum.151129

    Article  PubMed  Google Scholar 

  32. 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. https://doi.org/10.1016/j.semarthrit.2012.05.001

    Article  CAS  PubMed  Google Scholar 

  33. Hersh AO, Trupin L, Yazdany J, Panopalis P, Julian L, Katz P et al (2010) Childhood-onset disease as a predictor of mortality in an adult cohort of patients with systemic lupus erythematosus. Arthritis Care Res (Hoboken) 62(8):1152–1159. https://doi.org/10.1002/acr.20179

    Article  Google Scholar 

  34. Ambrose N, Morgan TA, Galloway J, Ionnoau Y, Beresford MW, Isenberg DA et al (2016) Differences in disease phenotype and severity in SLE across age groups. Lupus 25(14):1542–1550. https://doi.org/10.1177/0961203316644333

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  35. Lopes SRM, Gormezano NWS, Gomes RC, Aikawa NE, Pereira RMR, Terreri MT et al (2017) Outcomes of 847 childhood-onset systemic lupus erythematosus patients in three age groups. Lupus 26(9):996–1001. https://doi.org/10.1177/0961203317690616

    Article  CAS  PubMed  Google Scholar 

  36. Torrente-Segarra V, Salman Monte TC, Rua-Figueroa I, De Una-Alvarez J, Balboa-Barreiro V, Lopez-Longo FJ et al (2019) Relationship between damage and mortality in juvenile-onset systemic lupus erythematosus: cluster analyses in a large cohort from the Spanish Society of Rheumatology Lupus Registry (RELESSER). Semin Arthritis Rheum 48(6):1025–1029. https://doi.org/10.1016/j.semarthrit.2018.09.005

    Article  CAS  PubMed  Google Scholar 

  37. Torrente-Segarra V, Salman-Monte TC, Rua-Figueroa I, Del Campo V, Lopez-Longo FJ, Galindo-Izquierdo M et al (2020) Associated factors to serious infections in a large cohort of juvenile-onset systemic lupus erythematosus from Lupus Registry (RELESSER). Semin Arthritis Rheum 50(4):657–662. https://doi.org/10.1016/j.semarthrit.2020.05.016

    Article  PubMed  Google Scholar 

  38. Jin SY, Huang DL, Dang XQ, Yi ZW (2017) Lupus glomerulonephritis in 788 Chinese children: a multi-centre clinical and histopathological analysis based on 549 renal biopsies. Paediatr Int Child Health 37(4):286–291. https://doi.org/10.1080/20469047.2017.1309337

    Article  PubMed  Google Scholar 

  39. Ferreira J, Trindade VC, Espada G, Morel Z, Bonfa E, Magalhaes CS et al (2018) Epidemiology and management practices for childhood-onset systemic lupus erythematosus patients: a survey in Latin America. Clin Rheumatol 37(12):3299–3307. https://doi.org/10.1007/s10067-018-4254-4

    Article  PubMed  Google Scholar 

  40. Peterknecht E, Keasey MP, Beresford MW (2018) The effectiveness and safety of biological therapeutics in juvenile-onset systemic lupus erythematosus (JSLE): a systematic review. Lupus 27(13):2135–2145. https://doi.org/10.1177/0961203318804879

    Article  CAS  PubMed  Google Scholar 

  41. Brunner HI, Abud-Mendoza C, Viola DO, Calvo Penades I, Levy D, Anton J et al (2020) Safety and efficacy of intravenous belimumab in children with systemic lupus erythematosus: results from a randomised, placebo-controlled trial. Ann Rheum Dis 79(10):1340–1348. https://doi.org/10.1136/annrheumdis-2020-217101

    Article  CAS  PubMed  Google Scholar 

Download references

Funding

This study was supported by Scientific Research Talent Cultivation Fund of Meizhou People’s Hospital (PY-C2020009) and the Guangdong Provincial Key Laboratory of Precision Medicine and Clinical Translational Research of Hakka Population (2018B030322003).

Author information

Author notes

  1. Wengen Li and Sudong Liu Contributed equally to this work.

Authors and Affiliations

  1. Rheumatology and Immunology Department, Meizhou People’s Hospital (Huangtang Hospital), Meizhou Hospital Affiliated To Sun Yat-Sen University, No. 63 Huangtang Road, Meijiang District, Meizhou, 514031, People’s Republic of China

    Wengen Li & Chao Chen

  2. Clinical Core Laboratory, Meizhou People’s Hospital (Huangtang Hospital), Meizhou Hospital Affiliated To Sun Yat-Sen University, Meizhou, 514031, People’s Republic of China

    Sudong Liu

  3. Guangdong Provincial Key Laboratory of Precision Medicine and Clinical Translational Research of Hakka Population, Meizhou, 514031, People’s Republic of China

    Sudong Liu

  4. Pediatrics Department, Meizhou People’s Hospital (Huangtang Hospital), Meizhou Hospital Affiliated To Sun Yat-Sen University, Meizhou, 514031, People’s Republic of China

    Liuming Zhong

Authors
  1. Wengen Li
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Sudong Liu
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Liuming Zhong
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Chao Chen
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

WL conceived and designed the experiments, and wrote the manuscript; SL, CC, and LZ recruited subjects and collected clinical data. CC and LZ conducted the laboratory testing. WL and SL helped to analyze the data. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Wengen Li.

Ethics declarations

Ethics approval and consent to participate

The study was approved by the Ethics Committee of Meizhou People’s Hospital, Meizhou Hospital Affiliated to Sun Yat-sen University.

Disclosures

None.

Additional information

Publisher's note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Li, W., Liu, S., Zhong, L. et al. Clinical and laboratory features, disease activity, and outcomes of juvenile systemic lupus erythematosus at diagnosis: a single-center study from southern China. Clin Rheumatol 40, 4545–4552 (2021). https://doi.org/10.1007/s10067-021-05784-7

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-021-05784-7

Keywords

Search

Navigation