Clinical profiles in pediatric systemic lupus erythematosus: a retrospective study

Year 2024, Volume: 6 Issue: 1, 29 - 37, 15.01.2024

Aylin Kılınç Uğurlu , Sare Gülfem Özlü , Evrim Kargın Çakıcı , Özlem Erdoğan , Gülay Demircin , Ayşe Öner , Mehmet Bülbül

https://doi.org/10.38053/acmj.1379422

Cited By: 1

Abstract

Aims: This study aimed to analyse clinical and laboratory findings, prognosis, and survival of systemic lupus erythematosus (SLE) patients, differentiating according to gender, pubertal status, and renal involvement.
Methods: Ninety-six pediatric SLE patients, diagnosed using ACR criteria, were retrospectively analyzed. Inclusion criteria comprised age under 18, meeting at least four ACR criteria, and six months of monitoring. Data encompassed demographics, symptoms, diagnosis, organ involvement, autoantibodies, treatment, prognosis, and survival. Categorization was based on gender and pubertal status. Renal biopsies followed WHO-ISN classification, with asymptomatic findings termed “silent lupus nephritis.” Biopsied patients were divided into proliferative and non-proliferative lupus nephritis categories, excluding irreversible damage cases. Outcomes studied included remission, relapse, end-stage renal failure, and mortality.
Results: Among 96 participants, females constituted 82.3%, males 17.7%, resulting in a female-to-male ratio of 4.6:1. Mean age at diagnosis was 11.9 years, with 37 prepubertal (38.5%) and 59 pubertal (61.5%) cases. Oral-nasal ulcers (p=0.01) were more prevalent in males related to system involvement. Nephrotic syndrome prevalence increased from 21.6% in prepubertal to 44.1% in pubertal cases (p=0.025). Positive Anticardiolipin IgM antibodies decreased from 56.2% in prepubertal to 25.9% in pubertal cases (p=0.047). Type IV lupus nephritis was predominant, followed by Type II, in prepubertal and pubertal groups and both genders. Proliferative lupus nephritis showed higher rates of renal involvement (95.7% vs. 65.6%), nephrotic syndrome (46.8% vs. 21.9%), proteinuria (89.4% vs. 62.5%), hematuria (57.4% vs. 28.1%), elevated creatinine (43.5% vs. 9.7%), and low albumin (67.4% vs. 23.3%). Cases with proliferative lupus nephritis had higher neuropsychiatric involvement (36.2% vs. 12.5%), seizures (25.5% vs. 3.1%, p=0.008), and increased hemolytic anemia rates (78.7% vs. 56.2%, p=0.033). Thirteen had silent lupus nephritis, revealing various types through biopsy. All reported deaths occurred within the first five years, resulting in stable 91% survival rates at 5, 10, and 15 years.
Conclusion: This study provides insights into the clinical, prognostic, and survival characteristics of pediatric systemic lupus erythematosus (SLE), revealing notable patterns related to gender, pubertal development, and renal involvement. There is an association between proliferative lupus nephritis and renal involvement, nephrotic syndrome, and neuropsychiatric symptoms. Significantly, silent lupus nephritis highlights the complex renal implications, necessitating diligent surveillance for prompt intervention.

Keywords

Pediatric systemic lupus erythematosus (SLE), gender, pubertal status, renal involvement, proliferative lupus nephritis, silent lupus nephritis

References

• Bernatsky S, Joseph L, Pineau CA, Tamblyn R, Feldman DE, Clarke AE. A population-based assessment of systemic lupus erythematosus incidence and prevalence—results and implications of using administrative data for epidemiological studies. Rheumatol. 2007;46(12):1814-1818.
• Cassidy JT. Juvenile idiopathic arthritis. In Cassidy JT, Petty R. E. 2005.
• Salmon J, Pricop L, D’Agati V. Immunopathology of systemic lupus erythematosus. In: Hochberg M, Silman AK, Smolen J, et al. (eds.). Rheumatology. 6th ed. Elsevier Mosby, St Louis 2015:1052-1067.
• Zhu J, Wu F, Huang X. Age-related differences in the clinical characteristics of systemic lupus erythematosus in children. Rheumatol Int. 2013;33(1):111-115.
• Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheumatism. 1997;40(9):1725. doi:10.1002/art.1780400928
• Marshall WA, Tanner JM. Variations in pattern of pubertal changes in girls. Arch Dis Child. 1969;44(235):291-303.
• Churg J. Renal disease. Classific Atlas Glomerular Dis. 1995:155.
• Singh S, Saxena R, Palmer BF. Lupus nephritis. Am J Med Sci. 2009;337(6):451-460.
• Mina R, Von Scheven E, Ardoin SP, et al. Consensus treatment plans for induction therapy of newly diagnosed proliferative lupus nephritis in juvenile systemic lupus erythematosus. Arthritis Care Res. 2012;64(3):375-383.
• Cattran DC, Feehally J, Terence Cook H, et al. Kidney disease: improving global outcomes (KDIGO) glomerular diseases work group. KDIGO 2021 clinical practice guideline for the management of glomerular diseases. Kidney Int. 2021;100(4S):139-274.
• Lehman TJA, McCurdy DK, Bernstein BH, King KK, Hanson V. Systemic lupus erythematosus in the first decade of life. Pediatr. 1989;83(2):235-239.
• Smith CD, Cyr M. The history of lupus erythematosus: from Hippocrates to Osler. Rheumatic Dis Clin North Am. 1988;14(1):1-14.
• Pluchinotta FR, Schiavo B, Vittadello F, Martini G, Perilongo G, Zulian F. Distinctive clinical features of pediatric systemic lupus erythematosus in three different age classes. Lupus. 2007;16(8):550-555.
• Bader-Meunier B, Armengaud JB, Haddad E, et al. Initial presentation of childhood-onset systemic lupus erythematosus: a French multicenter study. J Pediatr. 2005;146(5):648-653.
• Walker SE. Estrogen and autoimmune disease. Clin Rev Allergy Immunol. 2011;40(1):60-65.
• Thabet Y, Mankaï A, Achour A, Sakly W, Trabelsi A, Harbi A. Systemic lupus erythematosus in children: a study about 37 Tunisian cases. J Clin Cell Immunol. 2014;5(192):2.
• Lukic A, Lukic IK, Malcic I, et al. Childhood-onset systemic lupus erythematosus in Croatia: demographic, clinical and laboratory features, and factors influencing time to diagnosis. Clin Experimen Rheumatol. 2013;31(5):803-812.
• Abdwani R, Abdalla E, Al-Zakwani I. Unique characteristics of prepubertal onset systemic lupus erythematosus. Int J Pediatr. 2019;2019:9537065.
• Qiu S, Zhang H, Yu S, et al. Clinical manifestations, prognosis, and treat-to-target assessment of pediatric lupus nephritis. Pediatr Nephrol. 2022;37(2):367-376. doi:10.1007/s00467-021-05164-y
• Nandi M, Mondal R. Renal involvement in childhood lupus: a study from Kolkata, India. Saudi J Kidney Dis Transplant. 2012;23(4):871.
• Chiang LL, Lin YT, Chan HY, Chiang BL. Differential manifestations of prepubescent, pubescent and postpubescent pediatric patients with systemic lupus erythematosus: a retrospective study of 96 Chinese children and adolescents. Pediatr Rheumatol. 2012;10(1):1-9.
• Perfumo F, Martini A. Lupus nephritis in children. Lupus. 2005;14(1):83-88.
• Khandelwal P, Govindarajan S, Bagga A. Management and outcomes in children with lupus nephritis in the developing countries. Pediatr Nephrol. 2023;38(4):987-1000. doi:10.1007/s00467-022-05769-x
• Boussetta A, Louati D, Jellouli M, et al. Lupus nephritis in Tunisian children: predictive factors of poor outcomes. Saudi J Kidney Dis Transplant. 2022;33(3):440-448. doi:10.4103/1319-2442.385968
• Rianthavorn P, Buddhasri A. Long-term renal outcomes of childhood-onset global and segmental diffuse proliferative lupus nephritis. Pediatr Nephrol. 2015;30(11):1969-1976.
• Hui-Yuen JS, Imundo LF, Avitabile C, Kahn PJ, Eichenfield AH, Levy DM. Early versus later onset childhood-onset systemic lupus erythematosus: clinical features, treatment and outcome. Lupus. 2011;20(9):952-959.
• Huang JL, Yeh KW, Yao TC, et al. Pediatric lupus in Asia. Lupus. 2010;19(12):1414-1418.
• Hiraki LT, Benseler SM, Tyrrell PN, Hebert D, Harvey E, Silverman ED. Clinical and laboratory characteristics and long-term outcome of pediatric systemic lupus erythematosus: a longitudinal study. J Pediatr. 2008;152(4):550-556.
• Wu J-Y, Yeh K-W, Huang J-L. Early predictors of outcomes in pediatric lupus nephritis: focus on proliferative lesions. In: Seminars in Arthritis and Rheumatism. Vol 43. Elsevier; 2014:513-520.
• Duran E, Tolga Y, Taghiyeva A, Bilgin E, Ar M, Ertenli AI. Differences and similarities of proliferative and non-proliferative forms of biopsy-proven lupus nephritis : single centre, cross-disciplinary experience. Lupus. 2022;31(9):1147-1156. doi:10.1177/09612033221106305
• Mannemuddhu SS, Shoemaker LR, Bozorgmehri S, et al. Does kidney biopsy in pediatric lupus patients “complement” the management and outcomes of silent lupus nephritis? lessons learned from a pediatric cohort. Pediatr Nephrol. 2023;38(8):2669-2678. doi:10.1007/s00467-022-05859-w
• González-Crespo MR, López-Fernández JI, Usera G, Poveda MJ, Gómez-Reino JJ. Outcome of silent lupus nephritis. In: Seminars in Arthritis and Rheumatism. Vol 26. Elsevier; 1996:468-476.
• Samanta M, Nandi M, Mondal R, et al. Childhood lupus nephritis: 12 years of experience from a developing country’s perspective. Eur J Rheumatol. 2017;4(3):178-183. doi:10.5152/eurjrheum.2017.16117
• Listiyono F, Murni I, Sumadiono S, Satria C. Predictors of mortality in children with systemic lupus erythematosus. Paediatr Indonesiana. 2019;59(1):1-6. doi:10.14238/pi59.1.2019.1-6