Abstract: SA-PO680
Initial Steroid Resistance Is Not Predictor of a Worst Prognosis in Children with Nephrotic Syndrome Sensitive to Calcineurin Inhibitors
Session Information
- Pediatric Glomerular Disease
November 09, 2019 | Location: Exhibit Hall, Walter E. Washington Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Pediatric Nephrology
- 1700 Pediatric Nephrology
Authors
- Morello, William, Pediatric Nephrology, Dialysis and Transplant Unit, Fondazione IRCCS Ca'' Granda Ospedale Maggiore Policlinico Milano, Milan, Italy
- Narang, Yashika, Pediatric Nephrology, Dialysis and Transplant Unit, Fondazione IRCCS Ca'' Granda Ospedale Maggiore Policlinico Milano, Milan, Italy
- Ghio, Luciana, Pediatric Nephrology, Dialysis and Transplant Unit, Fondazione IRCCS Ca'' Granda Ospedale Maggiore Policlinico Milano, Milan, Italy
- Montini, Giovanni, Pediatric Nephrology, Dialysis and Transplant Unit, Fondazione IRCCS Ca'' Granda Ospedale Maggiore Policlinico Milano, Milan, Italy
Background
Children with steroid resistant nephrotic syndrome (SRNS) have a high risk of progressing to end-stage renal disease (ESRD). While Calcineurin inhibitors (CNIs) effectively induce remission in >30% patients, long-term management of SRNS presents a significant clinical challenge. Very little is known about the evolution of SRNS in children, prognosis and the potential role of cortisteroid for relapse(s).
Methods
We performed a retrospective observational study of all paediatric patients treated with CNI for SRNS at our institution between 2008 and 2018. The data was collected about remissions achieved with CNIs, subsequent relapses, their treatment and remission, and long-term renal function.
Results
54 patients were included (41% males), with a median age of 12.5 years. After a median follow-up of 49 months, 38/54 patients (70.37%) achieved remission with CNIs, while the remaining 16 patients (29.63%) were unresponsive.18/38 patients (47.4%) had one or more relapse(s), (range:1-7). The total number of relapses was 48, out of which, 46 (95.8%) achieved remission. Of these, 38/46 (82.60%) were treated with corticosteroids only, 2/46 were treated with an increased dose of CNI only (>50% of initial dose), 5/46 (10.87%) were treated with both increased CNI dose and corticosteroids, and 1 patient achieved remission with mesenchymal stromal cell infusion. In total, 43 relapses were treated with steroids and 41 of these achieved a new remission. 2/48 relapses did not achieve remission despite different lines of treatment, but none had an impairment of renal function.
Conclusion
SRNS patients responsive to CNIs appear to have a good long-term prognosis, with no patient developing chronic kidey disease after a median follow-up of 49 months. 41/43 (95.3%) relapses were sensitive to corticosteroid therapy, despite the patient being SRNS at the onset of the disease. Therefore, corticosteroids can be considered an option for relapses of SRNS patients sensitive to CNIs. Steroid-sensitive and steroid-resistant NS responsive to CNIs are essentially a part of the spectrum of the same disease.