What’s the Scope of Nephritis in Pediatric Patients With IgA Vasculitis?

What’s the Scope of Nephritis in Pediatric Patients With IgA Vasculitis? 150 150 Jessica Nye, PhD

A multicenter study from the Pediatric Nephrology Research Consortium found that many pediatric patients with IgA vasculitis nephritis (IgAVN) are treated with immunosuppressive (IS) agents, and some had evidence of chronic kidney disease as early as 12 months after presentation.


IgA vasculitis (IgAV) is the most common vasculitis in children, usually presenting with typical purpuric rash, abdominal pain and arthritis. Up to half of children have kidney involvement. IgAVN has a heterogeneous presentation among children, and there remains a lack of consensus on how best the manage pediatric IgAVN.

“Like many other kidney diseases, IgAVN has many variables and genetic predisposition plays a role in its development and possibly its outcome,” says Mahmoud Kallash, MD, a nephrologist in the Section of Nephrology and Hypertension at Nationwide Children’s Hospital. “When a patient presents with an acute or subacute kidney disease, we often want to tackle it in a timely and sometimes, in an aggressive way. Current guidelines recommend supportive therapy for three to six months before initiation of IS agents, while our study showed that IS agents are used more commonly than supportive care. So, there is currently a disconnection between guidelines and what many nephrologists think patient’s care should be.”

To gain a better understanding of how patient presentation and treatment relate with 12-month outcomes, Dr. Kallash and colleagues reviewed records of 128 patients aged <21 years with IgAVN cared for at seven centers in the United States. The study was published in Pediatric Nephrology.

IgAVN presentation was variable. Most patients presented with hematuria and proteinuria (64%) with a urine protein/creatinine (UPC) ratio between 0.01-36.4 mg/mg and estimated glomerular filtration rate (eGFR) between 22-181 mL/min/1.73 m2. Acute kidney injury (AKI) was observed among 15%, and 6% had nephrotic syndrome.

Treatment for IgAN also varied. A third of patients were treated with steroids alone, a third received IS agents plus steroid therapy, and a quarter were treated with angiotensin-converting enzyme inhibitors (ACEi) as monotherapy or in combination with an IS agent. Anti-metabolite agents were the most common IS agents and the duration of therapy ranged between two and 84 weeks. Patients who received supportive care had significantly lower UPC at baseline.

At 12 months, 82 patients had normal UPC with no proteinuria (data missing in 39 subjects) and 102 patients had normal kidney function based on eGFR (data missing in 19 subjects). Overall, there was no significant difference between treatment groups for proteinuria or kidney function at 12 months.

“In our study, patients who received IS agents had more significant disease than the patients who got supportive care. So, it is difficult to draw conclusions about these patterns of treatment without a randomized or controlled study comparing both arms,” says Dr. Kallash, who is also associate professor of pediatrics at The Ohio State College of Medicine.

Kidney biopsy was performed on 69 patients, and there was no consensus on a threshold to perform kidney biopsy. More biopsies were performed among older patients and those with higher UPC at presentation. Crescents were observed in just over half of biopsies. Interestingly, crescents were more common in older patients and the severity of IgAVN on kidney pathology did not correlate with the degree of proteinuria or kidney function at baseline.

“Going forward, we should have a more systemic and evidence-based approach for treating this common disease” said Dr. Kallash. “A controlled study is needed. We need to evaluate different treatment strategies among patients with a similar degree of injury to truly know what the best treatment option for them is.”



Kallash M, Vogt BA, Zeid A, Khin E, Najjar M, Aldughiem A, Benoit E, Stotter B, Rheault M, Warejko JK, Daga A. The scope of treatment of pediatric IgA vasculitis nephritis and its outcome: a Pediatric Nephrology Research Consortium study. Pediatric Nephrology. Published online March 2, 2022. doi:10.1007/s00467-022-05496-3

About the author

Jessica Nye, PhD, is a freelance science and medical writer based in Barcelona, Spain. She completed her BS in biology and chemistry and MS in evolutionary biology at Florida State University. Dr. Nye studied population genetics for her doctorate in biomedicine at University of Pompeu Fabra. She conducted her postdoctoral research on the inheritance of complex traits at the Autonomous University of Barcelona.