41 - Post-Transplant Recurrence of Focal Segmental Glomerulosclerosis (FSGS): Challenges of Phenotype Across the Lifespan
Saturday, April 23, 2022
3:30 PM – 6:00 PM US MT
Poster Number: 41 Publication Number: 41.235
Eloise Salmon, University of Michigan Medical School, Ann Arbor, MI, United States; Richard eikstadt, University of Michigan Medical School, Ann Arbor, MI, United States; Hailey Desmond, University of Michigan Medical School, Ann Arbor, MI, United States; John C. Magee, University of Michigan Medical School, Ann Arbor, MI, United States; Jeffrey L. Platt, University of Michigan Medical School, Ann Arbor, MI, United States; David kershaw, University of Michigan Medical School, ann arbor, MI, United States; Debbie S. Gipson, University of Michigan Medical School, Ann Arbor, MI, United States
Clinical Assistant Professor University of Michigan Medical School Ann Arbor, Michigan, United States
Background: Recurrence of focal segmental glomerulosclerosis (FSGS) or minimal change disease (MCD) after kidney transplant has significant morbidity which can include graft loss. Reported rates of and risk factors for recurrence have varied, and studies have not included children consistently. A challenge in determining recurrence incidence in any age group is defining population at risk. Adaptive FSGS (attributed to mismatch between metabolic load and glomerular capacity, as in obesity) and monogenic FSGS considered unlikely to recur, but primary FSGS has a notable recurrence risk and can occur in patients with coexisting drivers for adaptive FSGS.
Objective: To describe pre- and post-transplant course of patients with FSGS and MCD across the lifespan with attention to risk factors for adaptive FSGS.
Design/Methods: In this retrospective cohort study, we used our center’s transplant registry to identify patients who received a kidney transplant and had a diagnosis of FSGS or MCD. Automated extraction from electronic health records (EHR) and manual chart review confirmed accuracy of diagnosis and collected key clinical features, including age of native disease onset, co-existing adaptive factor (obesity, hypertension, single kidney, prematurity), time to end stage kidney disease, and time to recurrence post-transplant.
Results: From 2011 - 2020, 143 patients with FSGS or MCD received a transplant at our center. Of these, 65 included in further analysis. The table summarizes features of the included patients as well as exclusion criteria for the remainder, then compares features of included patients with/without a clinical characteristic potentially, but not definitively, indicative of adaptive FSGS. Eight patients had genetic testing; five excluded for monogenic causes, the three included patients had no variants identified.Conclusion(s): Precise phenotyping to determine rates of and risk factors for FSGS recurrence after kidney transplant requires manual chart review to augment automated EHR extraction. In this small study, the presence of an adaptive risk factor was associated with a lower, but non-zero, risk of recurrence; the differences in time to recurrence suggest distinct clinical entities. Genetic testing infrequently sent. These data highlight potential areas of imprecision in labeling primary versus adaptive FSGS in registry classification schema. Pursuing well-designed multi-center collaboratives with robust phenotyping (including -omics) is critical to accelerate research focused on disease subtypes and mechanisms to improve care for children with FSGS and MCD undergoing kidney transplant. Clinical Features of Contemporary Cohort of Kidney Transplant Recipients with FSGS or MCD as the Cause of Native Kidney Disease