Diagnostic complications in the debut of systemic lupus erythematosus in children
Keywords:children, lupus nephritis, renal failure, systemic lupus erythematosus
Systemic lupus erythematosus (SLE) is an autoimmune multisystem disease that is more severe in children than in adults. The mortality of patients with SLE without treatment reaches 95.3% during the first 5 years from the onset of the disease. SLE develops more often in women. The ratio between girls and boys affected by SLE in prepubertal age is approximately 4.5:1 - 5:1, and in pubertal age - 9:1 - 10:1.
Purpose - to show, on the basis of clinical observation, the difficulties of diagnosis and treatment in the debut of SLE in children.
Clinical case. A 16-year-old child was treated in the children’s department of Road Hospital No. 1 with a diagnosis of «acute glomerulonephritis with leading incomplete nephrotic syndrome, hematuria, hypertension, tubulointerstitial component; a period of extensive clinical manifestations, with impaired kidney function. Postazotemic anemia. Secondary myelosuppression». She was admitted with complaints of swelling of the legs and face, changes in urine tests (proteinuria 1.65 g/day, leukocyturia 45-50 in the field of vision, erythrocyturia 35-40-50 in the field of vision). She considered herself sick when she noticed swelling of the ankle joints. Three weeks before admission to the hospital, she suffered acute pharyngitis (negative antistreptolysin O titer). Allergological history is not burdensome. At 6 years old - chicken pox. The child was prescribed corticosteroid therapy (prednisolone 50 mg/day), against the background of which, in the 4th week, the creatinine level normalized, but hypoproteinemia, elevated cholesterol, and proteinuria persisted, which indicated the lack of effect from CS therapy. Cytostatic therapy was started (leukeran - 8 mg/day (3 weeks); 4 mg/day - 3 weeks). Despite complex therapy, the child developed complete nephrotic syndrome, kidney failure six months later. During the examination of the patient, antinuclear antibodies with a titer >80 were detected, which gave rise to the calculation of additional criteria points (17 points) in accordance with the diagnostic criteria for SLE proposed by the European Antirheumatic League (EULAR) and the American College of Rheumatology (ACR), 2019. A diagnosis was made: SLE. Lupus nephritis, nephrotic syndrome, period of extensive clinical manifestations. Arterial hypertension; anemia I st. severity; azotemia; 2-3 degree of activity of the pathological process.
Conclusions. SLE in children is difficult to diagnose, especially in the absence of clinical polymorphism and systemic lesions. It is advisable to use the criteria proposed by EULAR and ACR, 2019, which are the most sensitive and specific in diagnosis. Treatment of patients with LN is individualized; the use of different treatment schemes depends on the severity of the pathological process. Early diagnosis of kidney damage in SLE using the criteria proposed by EULAR and ACR, 2019, and biopsy will contribute to the timely determination of the tactics and strategy of the child’s management, and improve the prognosis of Lupus nephritis.
The research was carried out in accordance with the principles of the Helsinki Declaration. The informed consent of the patient was obtained for conducting the studies
No conflict of interests was declared by the authors.
Calvo-Rio V, Santos-Gomez M, Calvo I et al. (2017). Anti-Interleukin-6 Receptor Tocilizumab for severe Juvenile idiopathic arthritis - associated uveitis refractory to anti-tumor necrosis factor therapy: A Multicenterstudy of twenty- five patients. Arthritis Rheumatology. 69 (3): 668-676. https://doi.org/10.1002/art.39940; PMid:27696756
Groot N, Graeff N, Avcin T et al. (2017). European evidence - based recommendations for diagnosis and treatment of childhood - onset sestemic lupus erythematosus: the SHARE initiative. Ann. Rheum. Dis. 76 (11): 1788-1796. https://doi.org/10.1136/annrheumdis-2016-210960; PMid:28630236
Lewandowski LB, Schanberg LE, Thielman N, Phuti A, Kalla AA, Okpechi I et al. (2017). Severe disease presentation and poor outcomes among pediatric systemic lupus erythematosus patients in South Africa. Lupus. 26 (2): 186-194. https://doi.org/10.1177/0961203316660625; PMid:27488473 PMCid:PMC5290292
Lythgoe H, Lj M, Hedrich CM, Aringer M. (2022, Jan). Classification of systemic lupus erythematosus in children and adults. Clin Immunol. 234: 108898. Epub 2021 Nov 29. https://doi.org/10.1016/j.clim.2021.108898; PMid:34856381
Mai K et al. (2022). Kidney transplant outcomes in children and adolescents with systemic lupus erythematosus. Pediatr transplant. 26: 1. https://doi.org/10.1111/petr.14178; PMid:34687584
Markowitz GS, D'Agati VD. (2007). The ISN/RPS 2003 classification of lupus nephritis: An assessment at 3 years. Kidney international. 71 (6): 491-495. https://doi.org/10.1038/sj.ki.5002118; PMid:17264872
Mina R, Brunner HI. (2010). Pediatric lupus - are trere differences in presentation, genetics, response to therapy, and damage accrual compared with adult lupus? Rheum. Dis. Clin. North. Am. 36 (1): 53-80. https://doi.org/10.1016/j.rdc.2009.12.012; PMid:20202591 PMCid:PMC2837537
Mina R, von Scheven E, Ardoin SP et al. (2012). Consensus treatment plans for induction therapy of newly diagnosed proliferative lupus nephritis in juvenile systemic lupus erythematosus. Arthritis Care Res. 64 (3): 375-383. https://doi.org/10.1002/acr.21558; PMid:22162255 PMCid:PMC3457803
Ruperto N, Abud-Mendoza С, Viola DO et al. (2019). The Pluto Study: intravenous bilimumab in children with systemic lupus erythematosus. Ann. Rheum. Dis. 78: 74-75.
Trindade VC, Carneiro-Sampaio M, Bonfa E, Silva CA. (2021, Jul). An Update on the Management of Childhood-Onset Systemic Lupus Erythematosus. Paediatr Drugs. 23 (4): 331-347. Epub 2021 Jul 10. https://doi.org/10.1007/s40272-021-00457-z; PMid:34244988 PMCid:PMC8270778
Tucker IB, Uribe AG, Fernandez M et al. (2008). Adolescent onset of lupus results in more aggressive disease and worse outcomes: results of nestedmatched case-control study within LUMINA, a multiethnic US cohort (LUMINA LVII). Lupus. 17 (4): 314-322. https://doi.org/10.1177/0961203307087875; PMid:18413413 PMCid:PMC2818044
Copyright (c) 2023 Modern pediatrics. Ukraine
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
The policy of the Journal “MODERN PEDIATRICS. UKRAINE” is compatible with the vast majority of funders' of open access and self-archiving policies. The journal provides immediate open access route being convinced that everyone – not only scientists - can benefit from research results, and publishes articles exclusively under open access distribution, with a Creative Commons Attribution-Noncommercial 4.0 international license (СС BY-NC).
Authors transfer the copyright to the Journal “MODERN PEDIATRICS. UKRAINE” when the manuscript is accepted for publication. Authors declare that this manuscript has not been published nor is under simultaneous consideration for publication elsewhere. After publication, the articles become freely available on-line to the public.
Readers have the right to use, distribute, and reproduce articles in any medium, provided the articles and the journal are properly cited.
The use of published materials for commercial purposes is strongly prohibited.