Death in the developed world is rare but well documented, and mainly occurs from infectious and thrombotic complications.[4]Gordillo R, Spitzer A. The nephrotic syndrome. Pediatr Rev. 2009;30:94-104.
http://www.ncbi.nlm.nih.gov/pubmed/19255123?tool=bestpractice.com
[5]Eddy AA, Symons JM. Nephrotic syndrome in childhood. Lancet. 2003 Aug 23;362(9384):629-39.
http://www.ncbi.nlm.nih.gov/pubmed/12944064?tool=bestpractice.com
[37]International Study of Kidney Disease in Children. Minimal change nephrotic syndrome in children: deaths during the first 5 to 15 years' observation. Report of the International Study of Kidney Disease in Children. Pediatrics. 1984;73:497-501.
http://www.ncbi.nlm.nih.gov/pubmed/6709428?tool=bestpractice.com
In the developing world, despite the limited therapeutic facilities, half of nephrotic syndrome (NS) patients benefit from corticosteroids, but corticosteroid resistance has been shown to result in high mortality.[38]Bhimma R, Adhikari M, Asharam K, et al. The spectrum of chronic kidney disease (stages 2-5) in KwaZulu-Natal, South Africa. Pediatr Nephrol. 2008;23:1841-1846.
http://www.ncbi.nlm.nih.gov/pubmed/18548285?tool=bestpractice.com
Lack of resources, late referrals, and the high cost of kidney replacement therapy in these countries leads to poor outcome in chronic kidney disease due to all causes, including NS and minimal change disease (MCD).[38]Bhimma R, Adhikari M, Asharam K, et al. The spectrum of chronic kidney disease (stages 2-5) in KwaZulu-Natal, South Africa. Pediatr Nephrol. 2008;23:1841-1846.
http://www.ncbi.nlm.nih.gov/pubmed/18548285?tool=bestpractice.com
Remission of proteinuria after corticosteroid therapy has greater prognostic value than the precise kidney histology in relation to long-term outcome.
Transition to adulthood
Most children outgrow MCD and stop having relapses in their teenage years although more than 10% of childhood-onset corticosteroid-sensitive NS patients still experience relapses during adulthood.[2]Trautmann A, Boyer O, Hodson E, et al. IPNA clinical practice recommendations for the diagnosis and management of children with steroid-sensitive nephrotic syndrome. Pediatr Nephrol. 2023 Mar;38(3):877-919.
https://link.springer.com/article/10.1007/s00467-022-05739-3
http://www.ncbi.nlm.nih.gov/pubmed/36269406?tool=bestpractice.com
However, kidney function can be normal and overall morbidity low.[39]Ruth EM, Kemper MJ, Leumann EP, et al. Children with corticosteroid-sensitive nephrotic syndrome come of age: long-term outcome. J Pediatr. 2005;147:202-207.
http://www.ncbi.nlm.nih.gov/pubmed/16126050?tool=bestpractice.com
Corticosteroid-resistant NS has a poor kidney outcome, with 34% to 64% progressing to end-stage renal disease (ESRD) within 10 years of diagnosis.[40]Hjorten R, Anwar Z, Reidy KJ. Long-term outcomes of childhood onset nephrotic syndrome. Front Pediatr. 2016;4:53.
https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2016.00053/full
http://www.ncbi.nlm.nih.gov/pubmed/27252935?tool=bestpractice.com
Transition to an adult nephrologist is recommended for all children approaching adulthood.
Even in adults, MCD has a favourable prognosis with over 80% of patients achieving remission with corticosteroids for the initial episode.[3]Kidney Disease: Improving Global Outcomes (KDIGO) Glomerular Diseases Work Group. KDIGO 2021 clinical practice guideline for the management of glomerular diseases. Kidney Int. 2021 Oct;100(4s):S1-276.
https://www.kidney-international.org/article/S0085-2538(21)00562-7/fulltext
However, as seen in children, the majority of the adults who go into remission will relapse, requiring long-term immunosuppression, with significant morbidity because of side effects.[1]Vivarelli M, Massella L, Ruggiero B, et al. Minimal change disease. Clin J Am Soc Nephrol. 2017 Feb 7;12(2):332-45.
https://cjasn.asnjournals.org/content/12/2/332.long
http://www.ncbi.nlm.nih.gov/pubmed/27940460?tool=bestpractice.com
A minority of adults with MCD eventually develop focal segmental glomerulosclerosis, which can lead to eventual kidney failure.[1]Vivarelli M, Massella L, Ruggiero B, et al. Minimal change disease. Clin J Am Soc Nephrol. 2017 Feb 7;12(2):332-45.
https://cjasn.asnjournals.org/content/12/2/332.long
http://www.ncbi.nlm.nih.gov/pubmed/27940460?tool=bestpractice.com
[3]Kidney Disease: Improving Global Outcomes (KDIGO) Glomerular Diseases Work Group. KDIGO 2021 clinical practice guideline for the management of glomerular diseases. Kidney Int. 2021 Oct;100(4s):S1-276.
https://www.kidney-international.org/article/S0085-2538(21)00562-7/fulltext
In addition, adults with MCD tend to respond more slowly, with more than 25% of responders taking 3-4 months or longer to undergo complete remission.[25]Hogan J, Radhakrishnan J. The treatment of minimal change disease in adults. J Am Soc Nephrol. 2013 Apr;24(5):702-11.
http://www.ncbi.nlm.nih.gov/pubmed/23431071?tool=bestpractice.com