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Age of Onset and Disease Course in Biopsy-Proven Minimal Change Disease: An Analysis From the Cure Glomerulopathy Network
Journal article   Peer reviewed

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

Dhruti P. Chen, Carla Nester, Margaret E. Helmuth, Abigail R. Smith, Pietro A. Canetta, Isabelle Ayoub, Krzysztof Mucha, Mahmoud Kallash, Jeffrey B. Kopp, Rasheed Gbadegesin, …
American journal of kidney diseases, Vol.81(6), pp.695-706.e1
06/01/2023
DOI: 10.1053/j.ajkd.2022.11.012
PMCID: PMC10200745
PMID: 36608921
url
https://pmc.ncbi.nlm.nih.gov/articles/PMC10200745/pdf/nihms-1875260.pdfView
Open Access

Abstract

Adolescent- and adult-onset disease 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 Glomerulopathy Network (CureGN) and assessed predictors of rituximab response.Prospective, multicenter, observational study.CureGN participants with proven MCD on biopsy.Age at disease onset. Initiation of RAAS blockade and immunosuppression including rituximab during the study period.Relapse and remission, change in eGFR and kidney failure.Remission and relapse probabilities are estimated using Kaplan-Meier curves and gap time recurrent event models. Linear regression models were used for the outcome of change in estimated glomerular filtration rate (eGFR). Cox proportional hazards models were used to estimate the association between rituximab administration and remission.304 childhood (≤12 years old), 49 adolescent (13-17 years old), and 201 adult onset (≥18 years) participants were included with 2.7-3.2 years of follow-up after enrollment. Children had longer time to biopsy (238 days vs. 23 in adolescents, and 36 in adults, p0.001) and were more likely to have received therapy prior to biopsy. Children were more likely to be treated with immunosuppression but not RAAS blockade. The rate of relapse was higher in childhood-onset versus adult-onset participants (hazard ratio (HR) 1.69 (95% confidence interval (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)). eGFR loss in all groups was minimal. Children were more likely to remit after rituximab than adolescents and adults (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).CureGN was limited to biopsy-proven disease. Comparisons of childhood to non-childhood cases of MCD may be subject to selection bias, given that childhood cases who are biopsied may be limited to those patients who are least responsive to initial therapy.Among patients with MCD who underwent kidney biopsy, there were differences in the course (relapse and remission) of childhood-onset compared to adolescent and adult-onset disease, as well as rituximab response.
 Nephrology

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