Good outcomes with mycophenolate-cyclosporine-based induction protocol in children with severe proliferative lupus nephritis

Abstract

The outcomes of children with severe proliferative lupus nephritis (LN) were examined using a new mycophenolate and cyclosporine-based (MMF-CSA) induction protocol. Sixteen children with LN (WHO class III and IV), 31.3% of whom required dialysis at induction, were retrospectively studied. Median MMF dose was 942 mg/m( 2)/day. Thirteen patients (81%) with persistent proteinuria received CSA. Clinical and laboratory parameters were compared at pre-induction, 6 and 12 months. Treatment outcome was defined by Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), renal function, haematuria, proteinuria and serological markers (complements C3, C4 and anti-dsDNA). Comparing these parameters at induction, 6 months and 12 months, respectively, SLEDAI (25.4 +/- 8.7 versus 3.2 +/- 2.9 versus 2.9 +/- 2.8), serum C3 (47 +/- 21 versus 107 +/- 27 versus 111 +/- 38 mg/dl), C4 (12 +/- 14 versus 23 +/- 14 versus 22 +/- 11 mg/dl) and urine protein (6.97 +/- 7.09 versus 0.98 +/- 1.56 versus 0.21 +/- 0.13 g/ day/1. 73 m(2)) improved significantly (p < 0.05). Anti-dsDNA titres decreased in 73% by 6 and 12 months (p < 0.05). Complete renal remission was achieved in 7/16 (43.8%) at 6 months and 12/16 (75%) at 12 months, the rest achieving partial remission with no treatment failures. In conclusion, a combination MMF-CSA protocol is an effective therapeutic alternative for induction of children with severe proliferative LN, resulting in significant clinical and serological improvement with minimal adverse effects.

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

“…When combining the high-quality evidence from RCT for LN in adult-onset SLE with the evidence from smaller case series and observational cohort studies in cSLE, the expert group recommends MMF (1200-1800mg/m2/day, maximum 1500 mg twice daily) or I.V. CYC in combination with high-dose prednisone (1-2 mg/kg/day, maximum 60 mg/day) for induction treatment of proliferative LN in cSLE (10,(42)(43)(44)(45)(46)(47)(48)(49)(50)(51)(52)(53)(54)(55)(56)(57)(58)(59)(60). The dosing of I.V.…”

Systemic Lupus Erythematosus

“…206,207 Proteinuria in children is defined by a spot urine protein/creatinine (Up/c) of greater than 0.2 mg/mg creatinine or by a 24-hour urine collection containing protein greater than 4 mg/m 2 /hour. [240][241][242][243][244][245][246][247][248][249] However, there have been no large-scale randomized, controlled trials in pediatric LN; treatment primarily has been determined from the adult studies collection is necessary to eliminate false readings due to the possible contribution of orthostatic proteinuria. Given the cumbersome nature of 24-hour urine collection, and the possible unreliability in young or incontinent children, Up/c determination is a convenient and reliable method for evaluating and following LN in children and shows good correlation with 24-hour urine collection.…”

An Update on Treatment and Outcome of Lupus Nephritis in Children

“…Cyclosporin A Cyclosporin A (Cyc A) can also be used in LN, but there are only a few trials involving a small number of patients studying the effects of it on LN [4]. Aragon et al [31] showed that together with MMF, Cyc A has been an alternative agent in the induction therapy in children with severe LN. There are also studies in children showing its effectiveness as a treatment option in the treatment of refractory cases of LN, but with high relapsing rates after cessation of the therapy [32,33].…”