Abstract: FR-PO654
Successful Voclosporin Treatment of Lupus Nephritis in an Adolescent
Session Information
- Pediatric Nephrology - II
November 03, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Pediatric Nephrology
- 1900 Pediatric Nephrology
Authors
- Mongia, Anil K., SUNY Downstate Health Sciences University, New York City, New York, United States
- Hamed, Basant, SUNY Downstate Health Sciences University, New York City, New York, United States
- Vastardi, Maria-Anna, SUNY Downstate Health Sciences University, New York City, New York, United States
- Fernandez, Hilda E., Columbia University Irving Medical Center, New York, New York, United States
Introduction
Lupus nephritis (LN) flare is a common cause of kidney injury. Rituximab (RTX) is a widely employed treatment for children with LN flare, though Its use may be limited by severe hypersensitivity reaction. We report here a 17-year-old AA girl with LN flare who had severe hypersensitivity to RTX, who showed significant improvement of proteinuria after introduction of Voclosporin (VCS). We believe this is the first reported case of the use of VCS in an adolescent with LN.
Case Description
17 yo AA girl diagnosed with LN class V in 2016 after she presented with facial rash, proteinuria, joint stiffness, and weight loss. She was treated with Prednisone and mycophenolate mofetil leading to complete remission. In 2019, she developed increasing proteinuria with flare and required 2 cycles of RTX with remission of LN. Cyclophosphamide (CYC) was not used due to parental refusal. She remained on hydroxychloroquine, prednisone, enalapril and aspirin.
In late 2022, she had a symptomatic LN flare with hypoalbuminemia, edema, and serum creatinine of 0.5mg/dL. Repeat RTX infusion led to a severe hypersensitivity reaction characterized by chest pain, SOB and generalized rash which required epinephrine, antihistamine and O2. Due to severe anaphylaxis, it was decided not to use further RTX. VCS 23.7mg BID was initiated starting Feb 2023.
Results:
With the use of VCS, patient had rapid improvement in her serum albumin and reduction of proteinuria within 2 weeks, which has been maintained to 12 weeks. At last visit her serum albumin improved to 3.3 gm/dL from 2.1gm/dL and UPCR (mg/mg creatinine) improved to 1.4 from 7.5 at the start of VCS. Her lipid profile ( Total cholesterol, LDL, TG) at follow up improved to ( 157,96,113) from ( 284,197,162) at start of VCS. No other significant changes in BP, hematologic parameters or eGFR. No adverse events from the use of VCS.
Discussion
Treatment options in children with LN are limited and mostly involve RTX and calcineurin inhibitors. Severe RTX hypersensitivity in our patient precluded its use. We have shown significant improvement of proteinuria with use of VCS in pediatric age group, suggesting it may be a useful option especially if other medications like RTX are not effective or contraindicated. Further controlled trials should be done to evaluate safety and efficacy of VCS in children.