Steroid Use in Vasculitis: To Treat or Not to Treat:
Pamela Weiss, MD, MSCE
Henoch-Schönlein purpura (HSP) represents the most common forms of vasculitis, or inflamed blood vessels, in children, affecting approximately 10 out of 100,000 children each year. Causing abdominal and joint pain, rashes, and potentially chronic renal disease, healthcare providers usually see an upswing in the number of HSP cases in the early fall, often after a patient has experienced an upper respiratory infection.
However, despite accounting for half of the pediatric vasculitis cases in the United States, healthcare providers continue to debate the appropriate treatment strategy for children with this painful and potentially serious condition.
The main topic of disagreement has centered not only on how but if HSP should be treated, particularly with steroids that are not currently part of the standard of care for inpatient management of HSP.
New Center for Pediatric Clinical Effectiveness faculty member Pamela Weiss, MD, MSCE, Division of Rheumatology, conducted a groundbreaking study looking at the children who are at risk for more severe problems stemming from HSP in an effort to determine the effect of steroid therapy.
Conducting a systematic meta-analysis of HSP and corticosteroid efficacy, Dr. Weiss found that patients treated early with steroids experienced resolution of their severe abdominal pain in 24 hours and reduced their odds of developing chronic renal disease.
In a subsequent study she used the Pediatric Health Information Systems database to look at the ways in which children with HSP were being treated across the country, with particular attention to steroids, narcotics, and other pain medications. She found significant differences in the use of these medications across children’s hospitals.
“The discrepancy across the country on how kids were being treated with steroids set the stage for a large comparative effectiveness study,” says Dr. Weiss, whose comparative effectiveness research (CER) study assessed the risk of surgery, abdominal imaging, and need for intravenous nutrition in children who did and did not receive steroids during the first days of hospitalization. She and her colleagues demonstrated that children who received steroids fared significantly better in regards to these outcomes. This CER study was recently published in Pediatrics.
“Data from these studies provide evidence to support treatment with steroids early in the course of HSP for those sick enough to require hospitalization,” says Dr. Weiss. “Future studies to further improve these patients’ outcomes, particularly the chronic renal disease, will need to focus on predictive models, risk stratification, and trials of additional immunomodulatory or renal-protective treatments.”
Dr. Weiss has extended her CER endeavors to a form of juvenile arthritis called enthesitis-related arthritis (ERA). Poorly controlled ERA can cause growth disturbances, joint contractures, joint destruction, and functional limitation in children. A subset of children with ERA will progress to ankylosing spondylitis, a condition characterized by spinal and back pain, stiffness, and eventual fusion of the vertebra.
There are a variety of drugs used to control the inflammation from ERA but, similar to HSP, there is also significant disagreement on which combinations of drugs work best.
“In the juvenile arthritis realm we have wonderful drugs, but most of them have the potential for significant toxicities,” says Dr. Weiss. “So it comes down to trying to provide the best treatment with the least amount of toxicity.”
