70 - Management & Outcomes of End-Stage Kidney Disease in Childhood Lupus Nephritis: A Collaboration of the Pediatric Nephrology Research Consortium and Childhood Arthritis & Rheumatology Research Alliance

Sunday, April 30, 2023

3:30 PM – 6:00 PM ET

Poster Number: 70
Publication Number: 70.351

Brian R. Stotter, Washington University in St. Louis School of Medicine, St. Louis, MO, United States; Ellen Cody, Washington University in St. Louis School of Medicine, Saint Louis, MO, United States; Ankana Daga, Boston Children's Hospital, Boston, MA, United States; Laurence Greenbaum, Emory University School of Medicine, Atlanta, GA, United States; Minh Dien Duong, University of Louisville School of Medicine, Louisville, KY, United States; Alexandra Mazo, The Children's Hospital at Montefiore, Maria Fareri Children's Hospital at Westchester Medical Center, White Plains, NY, United States; Beatrice Goilav, The Children's Hospital at Montefiore, Bronx, NY, United States; Alexis Boneparth, Columbia University Vagelos College of Physicians and Surgeons, New York, NY, United States; Laura Hesemann, University of Missouri-Columbia School of Medicine, Columbia, MO, United States; Mahmoud Kallash, Nationwide Children's Hospital, Columbus, OH, United States; Ahmed Zeid, Nationwide Children's Hospital, Columbus, OH, United States; Wacharee Seeherunvong, University of Miami Leonard M. Miller School of Medicine, Miami, FL, United States; Rebecca R. Scobell, Childrens Hospital of Philadelphia, Philadelphia, PA, United States; Issa Alhamoud, University of Iowa Stead Family Children's Hospital, Iowa, IA, United States; Caitlin Carter, UC San Diego/Rady Children's Hospital, San Diego, CA, United States; Caroline E. Straatmann, LSU Health and Children’s Hospital New Orleans, New Orleans, LA, United States; Bradley P. Dixon, University of Colorado School of Medicine, Aurora, CO, United States; Jennifer C.. Cooper, Children's Hospital Colorado, Aurora, CO, United States; Deborah Levy, The University of Toronto, Toronto, ON, Canada; Hermine I. Brunner, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States; Manpreet K. Grewal, Children's Hospital of Michigan, Detroit, MI, United States; Rossana Baracco, Central Michigan University College of Medicine, Children’s Hospital of Michigan, Detroit, MI, United States; Tanya Pereira, New York Medical College, Valhalla, NY, United States; Scott E. Wenderfer, University of British Columbia Faculty of Medicine, Vancouver, BC, Canada; Priya S. Verghese, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, United States

Presenting Author(s)

BS

Brian R. Stotter, MD (he/him/his)

Assistant Professor of Pediatrics
Washington University in St. Louis School of Medicine
St. Louis, Missouri, United States

Co-Author(s)

RN

Raoul Nelson, MD, PhD

Background: Lupus nephritis (LN) is a rare but important cause of end-stage kidney disease (ESKD) in children. Adult LN patients with kidney failure, particularly those receiving chronic dialysis, have a heightened risk of morbidity and mortality compared to those without kidney disease. The disease course of children with LN and ESKD remains poorly understood.

Objective:

Describe the clinical management and outcomes of patients with childhood LN who have reached kidney failure.

Design/Methods:

We performed a retrospective chart review of childhood LN patients with ESKD at 17 pediatric institutions between 2010 and 2019. Outcomes of interest included time on dialysis, lupus flare rates on dialysis and post-transplant, acute rejection rates, development of donor-specific antibodies (DSA), and patient mortality. Univariate logistic regression was used to identify factors predictive of lupus flare on dialysis and after transplantation, acute transplant rejection, and de novo DSA development.

Results:

Of the 59 included patients with childhood LN and ESKD (mean age 16.4 +/- 2.9 years, 89% female), kidney failure developed in 67% after a refractory lupus flare with dialysis-dependent AKI. Hemodialysis was the most common initial modality for patients with ESKD requiring dialysis (45/52, 87%); 7 patients received a preemptive kidney transplant. For those patients on dialysis, 17/52 (33%) patients had at least one lupus flare and there were 4 patient deaths (8%), all from major adverse cardiovascular events. Age at dialysis initiation, sex, and race were not significant risk factors for a lupus flare while on dialysis.

Twenty-four chronic dialysis patients (46%) received a kidney transplant, with a median dialysis vintage of 24.9 months (IQR: 12.5, 39.4). Mean follow-up of all 31 transplanted patients was 5.5 +/- 3.1 years. Post-transplant, 4/31 patients (13%) had a lupus flare and 14/31 patients (46%) developed de novo DSAs. Nineteen patients (61%) had acute transplant rejection, and 10/19 (53%) patients had ≥ 2 rejection episodes. Graft failure occurred in 8/31 (26%) of patients, and there were no patient deaths post-transplant. Age at kidney transplantation, sex, race, and dialysis vintage were not associated with occurrence of transplant rejection.

Conclusion(s): Children with LN who have ESKD commonly receive chronic dialysis for a prolonged period before transplantation. Even after initiation of chronic dialysis, one third of children with LN have flares that require additional immunosuppression. Transplanted children with LN are at higher risk for acute rejection and de novo DSA development than for recurrent LN.