Skip to main content
construction release_alert
Scholars@Duke will be undergoing maintenance April 11-15. Some features may be unavailable during this time.
cancel
Journal cover image

Age of Onset and Disease Course in Biopsy-Proven Minimal Change Disease: An Analysis From the Cure Glomerulonephropathy Network.

Publication ,  Journal Article
Chen, DP; Helmuth, ME; Smith, AR; Canetta, PA; Ayoub, I; Mucha, K; Kallash, M; Kopp, JB; Gbadegesin, R; Gillespie, BW; Greenbaum, LA; Kidd, J ...
Published in: Am J Kidney Dis
June 2023

RATIONALE & OBJECTIVE: Adolescent- and adult-onset minimal change disease (MCD) may have a clinical course distinct from childhood-onset disease. We characterized the course of children and adults with MCD in the Cure Glomerulonephropathy Network (CureGN) and assessed predictors of rituximab response. STUDY DESIGN: Prospective, multicenter, observational study. STUDY PARTICIPANTS: CureGN participants with proven MCD on biopsy. EXPOSURE: Age at disease onset, initiation of renin-angiotensin-aldosterone system (RAAS) blockade, and immunosuppression including rituximab during the study period. OUTCOME: Relapse and remission, change in estimated glomerular filtration rate (eGFR), and kidney failure. ANALYTICAL APPROACH: Remission and relapse probabilities were estimated using Kaplan-Meier curves and gap time recurrent event models. Linear regression models were used for the outcome of change in eGFR. Cox proportional hazards models were used to estimate the association between rituximab administration and remission. RESULTS: The study included 304 childhood- (≤12 years old), 49 adolescent- (13-17 years old), and 201 adult- (≥18 years) onset participants with 2.7-3.2 years of follow-up after enrollment. Children had a longer time to biopsy (238 vs 23 and 36 days in adolescent- and adult-onset participants, respectively; P<0.001) and were more likely to have received therapy before biopsy. Children were more likely to be treated with immunosuppression but not RAAS blockade. The rate of relapse was higher in childhood- versus adult-onset participants (HR, 1.69 [95% CI, 1.29-2.21]). The probability of remission was also higher in childhood-onset disease (HR, 1.33 [95%CI, 1.02-1.72]). In all groups eGFR loss was minimal. Children were more likely to remit after rituximab than those with adolescent- or adult-onset disease (adjusted HR, 2.1; P=0.003). Across all groups, glucocorticoid sensitivity was associated with a greater likelihood of achieving complete remission after rituximab (adjusted HR, 2.62; P=0.002). LIMITATIONS: CureGN was limited to biopsy-proven disease. Comparisons of childhood to nonchildhood cases of MCD may be subject to selection bias, given that childhood cases who undergo a biopsy may be limited to patients who are least responsive to initial therapy. CONCLUSIONS: Among patients with MCD who underwent kidney biopsy, there were differences in the course (relapse and remission) of childhood-onset compared with adolescent- and adult-onset disease, as well as rituximab response. PLAIN-LANGUAGE SUMMARY: Minimal change disease is a biopsy diagnosis for nephrotic syndrome. It is diagnosed in childhood, adolescence, or adulthood. Patients and clinicians often have questions about what to expect in the disease course and how to plan therapies. We analyzed a group of patients followed longitudinally as part of the Cure Glomerulonephropathy Network (CureGN) and describe the differences in disease (relapse and remission) based on the age of onset. We also analyzed rituximab response. We found that those with childhood-onset disease had a higher rate of relapse but also have a higher probability of reaching remission when compared with adolescent- or adult-onset disease. Children and all steroid-responsive patients are more likely to achieve remission after rituximab.

Duke Scholars

Altmetric Attention Stats
Dimensions Citation Stats

Published In

Am J Kidney Dis

DOI

EISSN

1523-6838

Publication Date

June 2023

Volume

81

Issue

6

Start / End Page

695 / 706.e1

Location

United States

Related Subject Headings

  • Urology & Nephrology
  • Treatment Outcome
  • Rituximab
  • Retrospective Studies
  • Recurrence
  • Prospective Studies
  • Nephrotic Syndrome
  • Nephrosis, Lipoid
  • Humans
  • Disease Progression
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Chen, D. P., Helmuth, M. E., Smith, A. R., Canetta, P. A., Ayoub, I., Mucha, K., … CureGN Consortium, . (2023). Age of Onset and Disease Course in Biopsy-Proven Minimal Change Disease: An Analysis From the Cure Glomerulonephropathy Network. Am J Kidney Dis, 81(6), 695-706.e1. https://doi.org/10.1053/j.ajkd.2022.11.012
Chen, Dhruti P., Margaret E. Helmuth, Abigail R. Smith, Pietro A. Canetta, Isabelle Ayoub, Krzysztof Mucha, Mahmoud Kallash, et al. “Age of Onset and Disease Course in Biopsy-Proven Minimal Change Disease: An Analysis From the Cure Glomerulonephropathy Network.Am J Kidney Dis 81, no. 6 (June 2023): 695-706.e1. https://doi.org/10.1053/j.ajkd.2022.11.012.
Chen DP, Helmuth ME, Smith AR, Canetta PA, Ayoub I, Mucha K, et al. Age of Onset and Disease Course in Biopsy-Proven Minimal Change Disease: An Analysis From the Cure Glomerulonephropathy Network. Am J Kidney Dis. 2023 Jun;81(6):695-706.e1.
Chen, Dhruti P., et al. “Age of Onset and Disease Course in Biopsy-Proven Minimal Change Disease: An Analysis From the Cure Glomerulonephropathy Network.Am J Kidney Dis, vol. 81, no. 6, June 2023, pp. 695-706.e1. Pubmed, doi:10.1053/j.ajkd.2022.11.012.
Chen DP, Helmuth ME, Smith AR, Canetta PA, Ayoub I, Mucha K, Kallash M, Kopp JB, Gbadegesin R, Gillespie BW, Greenbaum LA, Parekh RS, Hunley TE, Sperati CJ, Selewski DT, Kidd J, Chishti A, Reidy K, Mottl AK, Gipson DS, Srivastava T, Twombley KE, CureGN Consortium. Age of Onset and Disease Course in Biopsy-Proven Minimal Change Disease: An Analysis From the Cure Glomerulonephropathy Network. Am J Kidney Dis. 2023 Jun;81(6):695-706.e1.
Journal cover image

Published In

Am J Kidney Dis

DOI

EISSN

1523-6838

Publication Date

June 2023

Volume

81

Issue

6

Start / End Page

695 / 706.e1

Location

United States

Related Subject Headings

  • Urology & Nephrology
  • Treatment Outcome
  • Rituximab
  • Retrospective Studies
  • Recurrence
  • Prospective Studies
  • Nephrotic Syndrome
  • Nephrosis, Lipoid
  • Humans
  • Disease Progression