Clinical outcomes of childhood lupus nephritis: a single center’s experience

Abstract

This study retrospectively reviewed the medical records of children with lupus nephritis (LN) who were treated at Seoul National University Children's Hospital from 1986 to 2005 (mean duration 8.3+/-4.4 years). The records of 77 children (22 male and 55 female) were examined. The mean age at diagnosis was 11.9+/-3.0 years. The initial biopsy results revealed a WHO class IV classification for 60 (88.2%) of 68 biopsy proven cases. Of 77 patients, 67 (87.0%) responded initially to the high-dose corticosteroids with or without additional immunosuppressive therapy. Of the initial responders (67), 30 (44.8%) experienced at least one episode of proteinuric (24) or nephritic (6) flare. Thirteen patients (16.9%) progressed to either chronic renal failure (CRF) or end-stage renal disease (ESRD). Six (7.8%) patients died. A Kaplan-Meier estimate of patient survival and CRF-free survival rate was 95.4% and 88.7% at 5 years and 91.8% and 74.7% at 10 years, respectively. Multivariate analysis for class IV LN revealed male gender (P=0.029), initial hypertension (P=0.001) and absence of remission (P=0.002) to be prognostic factors predicting CRF. Glomerulosclerosis of 10% or more (P=0.005), nephritic flare (P=0.011), and presence of anti-phospholipid antibody (P=0.017) or syndrome (P=0.004) were also found to be independent risk factors for CRF. Cyclophosphamide pulse therapy failed to demonstrate superiority over other combined immunosuppressants used for the treatment of diffuse proliferative LN.

Predictors of Relapse and End Stage Kidney Disease in Proliferative Lupus Nephritis

“…Prior estimates of treatment resistance in children are more difficult to find as most of these studies report only long-term survival estimates. Treatment resistance was observed in 13% of a cohort of (n ϭ 77) Korean children with lupus nephritis and in 29% of a group of predominately (n ϭ 44) African American children with lupus nephritis (3,9). Treatment resistance was observed only among African Americans in our cohort.…”

“…Lupus nephritis affects 20 to 80% of juvenile onset lupus, and 10 to 50% of these individuals progress to end stage kidney disease (ESKD) (2)(3)(4)(5)(6). The prevalence and significance of disease relapse and impact of available treatments in pediatric patients with lupus nephritis are poorly understood.…”

Європейські Доказові Рекомендації По Діагностиці Та Лікуванню Люпус-Нефриту У Дітей: Ініціатива SHARE

“…Class III or IV LN, also known as proliferative LN, are the most common and severe forms of LN in cSLE (6,26,27,(39)(40)(41). Combination of class III or IV LN with class V LN is prevalent.…”

“…However, if a patient shows hardly any response within 3 months of induction treatment, it is generally accepted to change the principle induction agent. Renal flares have been reported to occur in up to 50% of cSLE patients during maintenance treatment (41,74,75). After excluding noncompliance to medication, restarting or increasing the dose of corticosteroids (oral prednisone or I.V.…”

Evaluation the effectiveness of intravenous cyclophosphamide treatment in children with lupus nephritis

“…NIH çalışmalarında, intravenöz siklofosfamid tedavisi ile renal yetmezlik insidansının diğer tedavi biçim-lerine göre önemli derecede düşük olduğu ve uzun süreli intravenöz siklofosfamid kullanımı ile %5 hastada SDBY geliştiği gösterilmiştir (20) . Buna karşılık, siklofosfamid tedavisinin renal sonuçlar açısından diğer tedavilere üstün olmadığını gösteren çalışmalar da mevcuttur (21,22) . Yapılan bazı çalışmalarda (23,24) intravenöz siklofosfamid sonrası SDBY gelişme oranı çalışmamıza göre daha yüksek bulunurken, intravenöz siklofosfamid kullanımı ile daha düşük oranlar bildirilen çalışmalar da mevcuttur (14,25) .…”