Treatment of Henoch-Schonlein purpura (HSP) Nephritis
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Abstract
Study to evaluate clinical and laboratory factors at initial presentation which can predict the course of patients with Henoch-Schonlein Purpura (HSP) nephritis, including duration of proteinuria and development of chronic kidney disease/end-stage renal disease, and to evaluate the association of various treatment regimens with outcomes such as time to remission of proteinuria and change in glomerular filtration rate in children with HSP nephritis.
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Description
Henoch-Schonlein purpura (HSP) is one of the most common forms of vasculitis in children, with an incidence of around 6-24 cases per 100,000 per year and with 90% of cases presenting before age ten. While many cases are self-limited, 30-50% of patients with HSP will develop renal involvement with about 20% having a nephritic or nephrotic syndrome, and 1-3% of patients progressing to end stage renal disease.
Currently there is a lack of evidence to guide treatment decisions for patients with HSP nephritis (HSPN), and therefore there is wide variation in treatment practices. Many small, uncontrolled, retrospective studies have attempted to describe various treatment regimens and their outcomes, but a large, multi-center, prospective trial has not been attempted. Current treatment recommendations are based on case series, retrospective analyses, and extrapolation of studies in patients with IgA nephropathy.
Thus, a prospective, multicenter clinical trial is needed to examine the outcomes in children with HSPN treated with different agents and protocols (e.g. ACE inhibition, corticosteroids, and other immunosuppressive agents). With the results of such a study, specific evidence-based guidelines could be created to guide the management of children with HSPN.Prior to initiation of such a trial, we need to better understand current treatment practice of HSPN. PEDSnet is the ideal platform to address our questions about treatment of HSP nephritis.Benefits of Using Learning Health System CorePediatric nephrology is a small field, and therefore multi-institutional research networks and learning health systems are essential for research, especially when working with rare diseases like HSPN. PEDSnet will allow us to access clinical data from a large number of patients treated at tertiary care centers. We will be able to analyze clinical and demographic data at presentation, indications for treatment, prescribing patterns at the various sites, and clinical and laboratory outcomes.
Hypothesis
Wide practice variation exists in the treatment of children with HSPN.
Study Aims
- To evaluate clinical and laboratory factors at initial presentation which can predict the course of patients with HSP nephritis, including duration of proteinuria and development of chronic kidney disease/end stage renal disease.
- To evaluate the association of various treatment regimens (e.g. ACE inhibitors, oral/IV steroids, other immunosuppressive agents, and conservative management without pharmaceutical agents) with outcomes such as time to remission of proteinuria and change in GFR in children with HSP nephritis.
Study Design
Retrospectively review all patients with HSP nephritis seen at the eight PEDSnet hospitals. Collect demographic and laboratory data from the time of initial presentation, renal biopsy findings, prescription history (ACE inhibitors, oral steroids, IV steroids, other immunosuppressive agents), and outcome data (duration of proteinuria, change in GFR, progression to ESRD, etc.). Perform statistical analyses to compare outcomes of patients receiving various combinations of therapies, with the goal to identify two or more potential treatments/treatment combinations associated with improved outcomes which can then be further analyzed in a prospective study.
Cohort Description
The cohort consisted of patients ages 0 to 21 years diagnosed with HSP nephritis, IgA vasculitis, IgA vasculitis with nephritis, or HSP, who were seen in a nephrology clinic at one of the participating centers between 2009 and 2019. Patients with normal urinalyses at the index visit are excluded.
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Related Publications
Stone HK, Mitsnefes M, Dickinson K, et al. 2023. “Clinical course and management of children with IgA vasculitis with nephritis.” Pediatric Nephrology. 38, 3721-3733. DOI: 10.1007/s00467-023-06023-8
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