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

CureGN Consortium

Research output: Contribution to journalArticlepeer-review

1 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)695-706.e1
JournalAmerican Journal of Kidney Diseases
Volume81
Issue number6
DOIs
StatePublished - Jun 2023

Bibliographical note

Funding Information:
Funding for the CureGN consortium is provided by U24DK100845 (formerly UM1DK100845), U01DK100846 (formerly UM1DK100846), U01DK100876 (formerly UM1DK100876), U01DK100866 (formerly UM1DK100866), and U01DK100867 (formerly UM1DK100867) from the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). Patient recruitment is supported by NephCure Kidney International . Dates of funding for first phase of CureGN was September 16, 2013 to May 31, 2019. DPC was supported by an NIH , National Institute of Diabetes and Digestive and Kidney Diseases Ruth L. Kirschstein National Research Service Award (NRSA) Institutional Research Training Grant ( T32DK007750 ; PI R.J. Falk). Dr Kopp is supported by the NIDDK Intramural Research Program. The funding agencies did not have a role in study design, data collection, analysis, reporting, or the decision to submit for publication.

Publisher Copyright:
© 2023 National Kidney Foundation, Inc.

Keywords

  • Adolescents
  • adults
  • age at disease onset
  • children
  • clinical phenotype
  • clinical trajectory
  • disease course
  • eGFR change
  • estimated glomerular filtration rate (eGFR)
  • minimal change disease (MCD)
  • nephrotic syndrome (NS)
  • prognosis
  • proteinuria
  • relapse
  • remission
  • rituximab

PubMed: MeSH publication types

  • Observational Study
  • Multicenter Study
  • Journal Article
  • Research Support, N.I.H., Intramural
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

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