Article Text
Abstract
Objective Lupus nephritis (LN) is a common and severe complication of childhood-onset SLE. Little is known about the long-term renal outcome of childhood-onset LN. We investigated predictive factors of end-stage kidney disease (ESKD) and chronic kidney disease (CKD) in a paediatric cohort of patients with LN.
Methods This is a retrospective multicentre study including patients with childhood-onset (<18 years) biopsy-proven LN. Clinical features were analysed at disease onset, time of kidney biopsy, 12 and 24 months after biopsy, and last follow-up. We investigated the prognostic significance of clinical, laboratory and histologic characteristics on the incidence of ESKD and CKD stage 3–5.
Results We included 73 patients with a median age of 14 years (IQR 11.5–17). At the time of kidney biopsy, most subjects displayed an aggressive disease: 2 (3%) children required haemodialysis, 27 (38%) had an estimated glomerular filtration rate (eGFR) <60 mL/min/1.73m2. The overall median eGFR was 70 mL/min/1.73m2 (IQR 43–96). Moreover, the median proteinuria was 4 g/24h (IQR 1.35–7.41), with 41 (59%) children displaying a nephrotic range proteinuria (i.e., >3 g/24h). The median follow-up was 13.3 years (IQR 4.7–25.4). A total of 10 patients (13.7%) reached ESKD, the majority of whom (12%) within 10 years from kidney biopsy; during the subsequent follow-up the incidence of ESKD stabilised (figure 1). At last follow-up, around 50% of patients displayed an eGFR <90 mL/min/1.73m2. A younger age at LN onset, a lower eGFR and central nervous system involvement at the time of kidney biopsy were identified as predictors of ESKD by a univariable Cox regression model (table 1). The same features were significantly associated with the occurrence of CKD stage 3–5 at last follow-up at a univariable logistic regression analysis.
Conclusion LN often presents with severe kidney function impairment and aggressive systemic involvement in children. In our cohort, whose follow-up was among the longest reported in the literature, 12% of patients reached ESKD within 10 years from kidney biopsy. Significant predictors of poor kidney outcome in the long term were a younger age at LN onset, a lower eGFR and the presence of neurological manifestations at the time of kidney biopsy.
Acknowledgements No funding was received for the present study.
This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/ .