Abstract
Background
The best treatment for IgAN is still debated. The trials NEFIGAN and NEFIGARD have demonstrated that TRF-budesonide (Nefecon) efficiently and safely reduced proteinuria in adults, leading to FDA approval of Nefecon for adult IgAN. In pediatric IgAN, an etiological treatment does not yet exist, and the main therapies remain RAAS inhibitors and oral steroids. To our knowledge, this is one of the few pediatric reports of TRF-budesonide therapy.
Case report—diagnosis/treatment
A 13-year-old boy underwent a kidney biopsy for recurrent macrohematuria and proteinuria, resulting in an IgAN diagnosis (MEST-C score M1-E1-S0-T0-C1). At admission, serum creatinine and UPCR were slightly increased. Three methylprednisolone pulses were performed, followed by prednisone and RAAS inhibitors therapy. However, after 10 months, macrohematuria became constant, and UPCR increased. A new kidney biopsy was performed, showing an increase in sclerotic lesions. Prednisone was discontinued, and a trial with IBD TRF-budesonide 9 mg/day started. One month later, macrohematuria episodes disappeared and UPCR decreased, with a stable kidney function. After 5 months, due to a reduction in morning cortisol levels and difficulty in drug provisioning, we started to wean TRF-budesonide by 3 mg every 3 months, with complete withdrawal after 1 year. During this period, episodes of macrohematuria dramatically decreased, and UPCR and kidney function were maintained stable.
Conclusion
Our case demonstrates that TRF-budesonide could be considered an effective second-line treatment in pediatric IgAN, particularly when a long course of steroids is necessary to control active inflammation. However, pediatric clinical trials to identify the correct dosage and tolerability of TRF-budesonide are urgently needed.
Similar content being viewed by others
References
Coppo R (2019) Pediatric IgA nephropathy in Europe. Kidney Dis 5:182–188. https://doi.org/10.1159/000495751
Suzuki H, Kiryluk K, Novak J et al (2011) The pathophysiology of IgA nephropathy. J Am Soc Nephrol 22:1795–1803. https://doi.org/10.1681/ASN.2011050464
Del Vecchio L, Rimoldi C, Pozzi C (2021) Nefecon (targeted-release formulation-budesonide) for the treatment of IgA nephropathy. Future Rare Dis 1:4. https://doi.org/10.2217/frd-2021-0013
FDA grants priority review for Nefecon for patients with IgA nephropathy (2021) Published online. Accessed April 28, 2021. https://prn.to/3t3pE8u.
Schwartz GJ, Muñoz A, Schneider MF, Mak RH, Kaskel F, Warady BA, Furth SL (2009) New equations to estimate GFR in children with CKD. J Am Soc Nephrol 20:629–637. https://doi.org/10.1681/ASN.2008030287
Venettacci O, Larkins N, Willis F (2018) Childhood IgA nephropathy successfully treated with targeted-release budesonide: a case report. J Paediatr Child Health 54:1403. https://doi.org/10.1111/jpc.14259
Śladowska-Kozłowska J, Galata B (2022) Abstracts of the 54th ESPN Annual Meeting, Ljubljana, Slovenia. Pediatr Nephrol 37:2803–2969. https://doi.org/10.1007/s00467-022-05680-5
Fellström BC, Barratt J, Cook H, Coppo R, Feehally J, de Fijter JW, Floege J, Hetzel G, Jardine AG, Locatelli F, Maes BD, Mercer A, Ortiz F, Praga M, Sørensen SS, Tesar V, Del Vecchio L, NEFIGAN Trial Investigators (2017) Targeted-release budesonide versus placebo in patients with IgA nephropathy (NEFIGAN): a double-blind, randomized, placebo-controlled Phase IIb trial. Lancet 389:2117–2127. https://doi.org/10.1016/S0140-6736(17)30550-0
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors declare no competing interests.
Additional information
Publisher’s note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.
About this article
Cite this article
Antonucci, L., Colucci, M., Emma, F. et al. A pediatric case of IgA nephropathy benefitting from targeted release formulation–budesonide. Pediatr Nephrol 38, 3849–3852 (2023). https://doi.org/10.1007/s00467-023-05968-0
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00467-023-05968-0