Measures in assessment of pediatric systemic lupus erythematosus: an experience of retrospective observational study

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Abstract


Systemic lupus erythematosus in children (juvenile-onset SLE, jSLE) is a multisystemic disease with an unpredictable course and a more severe phenotype compared to adults. The patterns of jSLE are extremely heterogeneous, so an enrollment to controlled studies may be rather complicated. Due to this problem and some additional ones, there are no standards for treatment of jSLE yet. The attending physician is fully responsible for the induction and maintenance therapeutical options including durability and aggressiveness.

Objectives: finding of jSLE individual course’s features prognostically connected with the disease outcome.

Methods: 45 children admitted to the SPbGPMU hospital with the systemic lupus erythematosus diagnosed at the age of 4-17 years were enrolled in this retrospective study. Primary SLE manifestations, the activity of disease according to SELENA-SLEDAI and ECLAM scales during initial treatment period and flares after it, the fact of remission achievement in 6 months were evaluated in each patient.

Results: a few organ involvements were considered to be connected with outcome’s characteristics, for example lupus nephritis and early disease oncet are unfavorable predictive factors. The positive connection of favorable outcome with cyclophosphamide, intravenous methylprednisolone and mycophenolate mofetil was found; the negative connection between initial disease activity and flares after induction treatment was also noticed.

Conclusion: the patient with initially high disease activity treated aggressively with high cumulative doses of cyclophosphamide, intravenous methylprednisolone and mycophenolate mofetil has more chances of the favorable outcome (the achievement of remission without further flares).


Ekaterina M. Kuchinskaya

Author for correspondence.
kuchinskaya.link@gmail.com
St. Petersburg State Pediatric Medical University
Russian Federation, St. Petersburg

Postgraduate Student, Department of Hospital Pediatrics

Vyacheslav G. Chasnyk

chasnyk@list.ru
St. Petersburg State Pediatric Medical University
Russian Federation, St. Petersburg

MD, PhD, Dr Med Sci, Professor, Head, Department of Hospital Pediatrics

Mikhail M. Kostik

kost-mikhail@yandex.ru
St. Petersburg State Pediatric Medical University
Russian Federation, St. Petersburg

MD, PhD, Associate Professor, Department of Hospital Pediatrics

  • Костик М.М., Снегирева Л.С., Дубко М.Ф., и др. Как распознать пациента с аутовоспалительным синдромом: Клинико-диагностические алгоритмы // Современная ревматология. – 2013. – № 3. – С. 14–20. [Kostik MM, Snegireva LS, Dubko MF, et al. How to identity a patient with autoinflammatory syndrome: Clinical and diagnostic algorithms. Sovremennaya revmatologiya. 2013;3:14-20. (In Russ.)]
  • Костик М.М., Кучинская Е.М., Абдурагимова Ф.Н., и др. Опыт применения ритуксимаба у детей с системной красной волчанкой: ретроспективное исследование серии случаев // Вопросы современной педиатрии. – 2016. – Т. 15. – № 3. – С. 295–300. [Kostik MM, Kuchinskaya EM, Abduragimova FN, et al. Experience in Rituximab Administration in Children with Systemic Lupus Erythematosus: a Retrospective Study of the Case Series. Voprosy sovremennoy pediatrii. 2016;15(3):295-300. (In Russ.)]. doi: 10.15690/vsp.v15i3.1567.
  • Кучинская Е.М., Костик М.М., Часнык В.Г. Современные основы формализованного описания течения системной красной волчанки у детей // Педиатр. – 2017. – Т. 8. – № 2. – С. 68–80. [Kuchinskaya EM, Kostik MM, Chasnyk VG. Measures in assessment of pediatric systemic lupus erythematosus. Pediatr. 2017;8(2):68-80. (In Russ.)]. doi: 10.17816/PED8268-80.
  • Arici ZS, Batu ED, Ozen S. Reviewing the Recommendations for Lupus in Children. Curr Rheumatol Rep. 2015;17:17-30. doi: 10.1007/s11926-014-0489-5.
  • Bertsias GK, Tektonidou M, Amoura Z, et al. Joint European League Against Rheumatism and European Renal Association-European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of adult and paediatric lupus nephritis. Ann Rheum Dis. 2012;71(11):1771-1782. doi: 10.1136/annrheumdis-2012-201940.
  • Brunner HI, Gladman DD, Ibañez D, et al. Difference in disease features between childhood-onset and adult-onset systemic lupus erythematosus. Arthritis Rheum. 2008;58(2):556-562. doi: 10.1002/art.23204.
  • Group KDIGOKGW. KDIGO clinical practice guideline for glomerulonephritis. Kidney Int. 2012;2:139-274.
  • Mok CC. Treat-to-target in systemic lupus erythematosus: are we there yet? Expert Rev Clin Pharmacol. 2016;9(5):675-680. doi: 10.1586/
  • 2016.1146589.
  • Mosca M, Bombardieri S. Assessing remission in systemic lupus erythematosus. Clin Exp Rheumatol. 2006;24(6):99-104.
  • Van Vollenhoven RF, Mosca M, Bertsias G, et al. Treat-to-target in systemic lupus erythematosus: recommendations from an international task force. Ann Rheum Dis. 2014;73(6):958-967. doi: 10.1136/annrheumdis-2013-205139.
  • Van Vollenhoven RF, Voskuyl A, Morand E. Remission in SLE: closing in on the target. Ann Rheum Dis. 2015;74:2103-2106. doi: 10.1136/annrheumdis-2015-208231.

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