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Study Confirms Urgent, Unmet Needs in Childhood-Onset Lupus

Experts identify lupus nephritis and neuropsychiatric disease among highest research priority areas.

The Childhood Arthritis and Rheumatology Research Alliance (CARRA) and Lupus Foundation of America (LFA) conducted a first-of-its-kind, multidisciplinary research effort on childhood-onset lupus to assess research priorities for addressing this life-threatening disease. Results from the study, “Research Priorities in Childhood-Onset Lupus: Results of a Multidisciplinary Prioritization Exercise” published in Pediatric Rheumatology, conclude that the highest areas needing research in childhood lupus are nephritis, clinical trials, biomarkers, neuropsychiatric disease, and refractory skin disease.

The study also revealed a strong need for multidisciplinary collaboration moving forward. When asked which sub-specialties in addition to rheumatology should collaborate in childhood lupus research, 92% suggested collaborating with nephrologists, 73% with dermatologists, and 68% with mental health specialists.

Children with lupus have more pervasive and life-threatening organ involvement compared to adults with lupus, and an estimated 6,000 US children and adolescents are currently living with the disease which has few treatment options and no cure. Over 250 members of CARRA, Midwestern Pediatric Nephrology Consortium (MWPNC) and Pediatric Dermatology Research Alliance (PeDRA) Connective Tissue Disease Group participated in the study.

Inflammation of the Kidneys

Nephritis (inflammation of the kidneys) was identified as the number one research priority. Up to 80% of children with lupus develop nephritis, which increases risk of kidney failure, cardiovascular disease and death. Lupus nephritis (LN) requires prolonged therapy, often with toxic medications, and complex treatment plans. Although there have been many advances over the last several decades and many people with lupus can enjoy good health and normal activities, LN is still a driving factor in increased complications, hospitalizations and mortality rates, especially in children versus adults with lupus. 

Cognitive Impairment

Lupus commonly affects the brain in children and can cause strokes as well as severely impair the ability to think through tasks. Neuropsychiatric disease (involvement of the nervous system) was also identified as a top challenge for clinicians and researchers treating children with lupus. Neuropsychiatric complications can have substantial negative impact on cognitive development, educational and vocational outcomes in children and adolescents with lupus. 

Up to 65% of children with lupus develop neuropsychiatric involvement over the course of their disease, with up to 85% developing neuropsychiatric involvement in the first two years from diagnosis.1 The burden, ranging from missed school or even school failure to major challenges with treatment adherence, can have an enormous impact on a child’s psychological development and  quality of life. Today, the treatment of childhood lupus is generally extrapolated from adult clinical trials data and optimal drug dosing, duration of therapy and outcomes measurement may differ for pediatric patients. 

Specific to nephritis and neuropsychiatric disease, important focus areas across all lupus experts were determining best treatments, biomarkers/pathophysiology, drug discovery/novel treatments, understanding long term outcomes, and refining provider reported quality measures. Of note, understanding long term outcomes was ranked highly important across every research area. 

“This study underscores the urgency for childhood lupus research. We are committed to investing in this area, and our partnership with CARRA as leaders in this field will bolster our progress as we work together to elevate childhood lupus research,” said Stevan Gibson, President and CEO, Lupus Foundation of America. “The findings from the study offer a roadmap to guide future research endeavors, so that the scope and devastation of lupus in children aligns with received funding.”

“This is the first published research prioritization effort among experts in childhood lupus, which highlights the lack of evidence that exists specific to this disease. There are important differences between adults and children, and significant knowledge gaps limit our understanding of the best treatments and long term outcomes in children with lupus,” said Aimee Hersh, Chair of the CARRA Systemic Lupus Erythematosus Committee. “This multidisciplinary approach to identifying research priorities emphasizes the need for collaboration for childhood lupus care and research. The results from this exercise will help set a research agenda moving forward.”


The study, Research Priorities in Childhood-Onset Lupus: Results of a Multidisciplinary Prioritization Exercise, appears in Pediatric Rheumatology.

  1. Systemic Lupus Erythematosus in Children and Adolescents, Pediatric Clinics of North America, Volume 59, Issue 2, April 2012