Abstract

The article presents a historical and prospective analysis of the clinical and paraclinical data of a cohort of children suffering from systemic lupus erythematosus (SLE) with kidney damage, observed in a single center for 21 year. The case histories of 62 patients (51 girls and 11 boys) with a diagnosis of SLE and lupus nephritis (LN) who were monitored and treated at the Byelorussian Center for Pediatric Nephrology and Renal Replacement Therapy of the 2nd Children's Clinical Hospital in Minsk in the period from 1998 to 2019 yrs. We evaluated the initial manifestations of the disease, age of the onset, time of diagnosis (from the first symptom to the diagnosis), clinical manifestations at the time of diagnosis, the duration of SLE and treatment at the time of kidneys biopsy, as well as the activity of the disease. During the observation period (from 3 months up to 12 years) the frequency of extrarenal and renal exacerbations was analyzed, and the activity, treatment and outcome were evaluated. Male gender and early debut are factors worsening the prognosis of the disease (55% of boys showed progression of CRF). In children, the disease proceeds with a high degree of activity, rapid multi-organ involvement, requires more aggressive therapy with prescription of several immunosuppressive agents, which increases the risk of infection. Nephritis is an unfavorable prognostic criterion and is crucial in determining the volume of therapy for SLE. Noncompliance in therapy, social status were factors of an unfavorable outcome in children. Timely diagnosis and aggressive intervention to suppress the activity of the disease should be used to prevent chronic irreversible damage. Children with SLE and kidney damage are at high risk for early development of cardiovascular disorders. Steroid-sparing strategies must be actively implemented in clinical practice in order to prevent severe side effects.

Highlights

  • Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by multisystem involvement and a chronic relapsing course [1, 2]

  • Analysis of demographic data shows that gender ratio in childhood onset SLE is different from adults: out of 62 children 11 were boys and male gender may act as an unfavorable prognostic factor (55% of boys showed progressive relapsing course of nephritis to end stage renal disease (ESRD))

  • Analysis of demographic data shows that gender ratio in childhood onset SLE is different from adults: out of 62 children 11 were boys (5,6: 1)

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Summary

Introduction

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by multisystem involvement and a chronic relapsing course [1, 2]. Childhood onset lupus includes patients with a debut of the disease under the age of 18 and has a number of features. The ratio of girls and boys (5-8: 1) is less pronounced compared with adults, the clinical phenotype is characterized by a higher activity and frequency of renal, hematological and neuropsychiatric symptoms, multi-organ lesion and worse prognosis [3, 4]. The purpose of this study is a historical and prospective analysis of the clinical and paraclinical data of a cohort of children with SLE, LN at the onset of the disease and with long-term follow-up, treatment and outcome to assess factors that affect the prognosis of the disease

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