Browsing by Subject "Membranous nephropathy"

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    Comparing adolescent glomerular disease clinical outcomes to the clinical outcomes in childhood, young adult, and adult-onset glomerular disease in the CureGN database
    (Springer, 2025) Garrity, Kelly; Putnam, Nathaniel; Kamil, Elaine S.; Massengill, Susan; Khalid, Myda; Srivastava, Rachana; Isaacs, Jaya; Salmon, Eloise; Pediatrics, School of Medicine
    Background: There is a lack of evidence to suggest that outcomes of adolescent and adult-onset glomerular disease differ. Still, most glomerular disease trials include adults but exclude adolescents. Methods: We designed a retrospective study using the CureGN database to compare individuals with adolescent-onset glomerular disease relative to individuals with older and younger age at onset. The two main outcomes were sustained proteinuria remission off immunosuppression treatment and composite eGFR decline. Results: Our data did not show a significant difference in sustained proteinuria remission off treatment or composite eGFR decline between adolescent onset glomerular disease and either childhood (age 5-12), young adult (age 20-29), or adult (age 30-39) onset glomerular disease. Having high-risk APOL1 alleles and hypertension at the time of study enrollment decreased the likelihood of achieving sustained proteinuria remission off treatment. While participants with minimal change disease and IgA nephropathy were similarly likely to achieve sustained proteinuria remission off treatment, participants with focal segmental glomerulosclerosis and membranous nephropathy were less likely to achieve sustained proteinuria remission off treatment compared to participants with minimal change disease. CKD stage, high-risk APOL1 alleles, hypertension stage, and education all significantly impacted the likelihood of progression to the composite eGFR decline outcome. Conclusions: Approximately 25% of each age cohort reached the composite eGFR decline outcome within 5 years. As more glomerular disease clinical trials become available, we must consider opening these trials to people with childhood and adolescent onset disease since like adults they are at high risk of progressive kidney function decline.
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    Treatment Patterns Among Adults and Children With Membranous Nephropathy in the Cure Glomerulonephropathy Network (CureGN)
    (Elsevier, 2019-12) O’Shaughnessy, Michelle M.; Troost, Jonathan P.; Bomback, Andrew S.; Hladunewich, Michelle A.; Ashoor, Isa F.; Gibson, Keisha L.; Matar, Raed Bou; Selewski, David T.; Srivastava, Tarak; Rheault, Michelle N.; Al-Uzri, Amira; Kogon, Amy J.; Khalid, Myda; Vento, Suzanne; Sanghani, Neil S.; Gillespie, Brenda W.; Gipson, Debbie S.; Wang, Chia-shi; Parsa, Afshin; Guay-Woodford, Lisa; Laurin, Louis-Philippe; Pediatrics, School of Medicine
    Introduction The 2012 Kidney Disease: Improving Global Outcomes (KDIGO) Clinical Practice Guidelines for Glomerulonephritis recommend that patients with membranous nephropathy (MN) at risk for progression receive immunosuppressive therapy (IST), usually after 6 months of observation. A cyclophosphamide (CYC) or calcineurin inhibitor (CNI)–based regimen is recommended as first-line IST. However, the extent to which KDIGO recommendations are adopted in practice remains largely unknown. Methods We evaluated prescribing practice among patients with primary MN (diagnosed 2010–2018) enrolled in the Cure Glomerulonephropathy Network (CureGN) cohort study. We also evaluated the availability of testing for phospholipase A2 receptor (PLA2R) in the contemporary era. Results Among 361 patients (324 adults and 37 children) with MN who were IST-naïve at biopsy and had at least 6 months of follow-up, 55% of adults and 58% of children initiated IST <6 months after biopsy. Of these, 1 in 5 had no indication for (i.e., urine protein-to-creatinine ratio [uPCR] <4 g/g) or an apparent contraindication to (i.e., an estimated glomerular filtration rate [eGFR] <30 ml/min per 1.73 m2) IST. As first-line IST, half of treated patients received either CYC (16% of adults; 0% of children) or a CNI (40% and 46%, respectively), whereas 1 in 5 received corticosteroid monotherapy (20% and 27%, respectively) and 1 in 6 rituximab (15% and 15%, respectively). More than 80% of surveyed centers had access to PLA2R testing. Conclusion These findings suggest that providers are not aware of, or lack confidence in, current KDIGO guidelines for MN. Treatment patterns observed in this cohort might critically inform the drafting of planned updates to KDIGO guidelines.