Principles of pediatric lupus nephritis in a prospective contemporary multi-center cohort

Author:

Vazzana Kathleen M1ORCID,Daga Ankana2,Goilav Beatrice3,Ogbu Ekemini A4ORCID,Okamura Daryl M5,Park Catherine6,Sadun Rebecca E7,Smitherman Emily A8,Stotter Brian R9,Dasgupta Abhijit10,Knight Andrea M11,Hersh Aimee O12,Wenderfer Scott E13ORCID,Lewandowski Laura B1,

Affiliation:

1. Lupus Genomics and Global Disparities Unit, Systemic Autoimmunity Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA

2. Division of Nephrology, Boston Children's Hospital, Boston, MA, USA

3. Division of Nephrology, The Children’s Hospital at Montefiore, Albert Einstein College of Medicine, Bronx, NY, USA

4. Department of Pediatrics, Johns Hopkins University, Division of Pediatric Allergy, Immunology and Rheumatology, Johns Hopkins University, Baltimore, MD, USA

5. Division of Nephrology, Seattle Children’s Hospital, Seattle, WA, USA

6. Division of Nephrology, Children’s National Hospital, Washington, DC, USA

7. Divsion of Rheumatology, Duke University, Durham, NC, USA

8. Division of Rheumatology, Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL, USA

9. Division of Pediatric Nephrology, Hypertension & Pheresis, Department of Pediatrics, Washington University School of Medicine, St. Louis, MO, USA

10. ARAASTAT, Germantown, MD, USA

11. Division of Pediatric Rheumatology, Hospital for Sick Children, Hospital for Sick Children, Toronto, Canada

12. Division of Pediatric Rheumatology, Department of Pediatrics, University of Utah, Salt Lake City, UT, USA

13. Division of Pediatric Nephrology, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, TX, USA

Abstract

Lupus nephritis (LN) is a life-threatening manifestation of systemic lupus erythematosus (SLE) and is more common in children than adults. The epidemiology and management of childhood-onset SLE (cSLE) have changed over time, prompting the need to reassess expected outcomes. The purpose of this study is to use the Childhood Arthritis and Rheumatology Research Alliance (CARRA) prospective registry to validate historical principles of LN in a contemporary, real-world cohort. After an extensive literature review, six principles of LN in cSLE were identified. The CARRA registry was queried to evaluate these principles in determining the rate of LN in cSLE, median time from cSLE diagnosis to LN, short-term renal outcomes, and frequency of rituximab as an induction therapy. Of the 677 cSLE patients in the CARRA registry, 32% had documented LN. Decline in kidney function was more common in Black cSLE patients than non-Black patients ( p = 0.04). Black race was associated with worse short-term renal outcomes. In short-term follow up, most children with LN had unchanged or improved kidney function, and end stage kidney disease (ESKD) was rare. Ongoing follow-up of cSLE patients in the CARRA registry will be necessary to evaluate long-term outcomes to inform risk, management, and prognosis of LN in cSLE.

Funder

Lupus Foundation of America

Publisher

SAGE Publications

Subject

Rheumatology

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