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IgA nephropathy and Henoch-Schönlein purpura nephritis
John T Sanders1, Robert J Wyatt
1Children's Foundation Research Center at Le Bonheur Children's Medical Center, Memphis, Tennessee, USA.
Insights
IgA nephropathy and Henoch-Schönlein purpura nephritis are kidney diseases in children. Research shows galactose-deficient IgA1 may aid diagnosis, and ACE inhibitors can reduce proteinuria.
Area of Science:
- Pediatric Nephrology
- Glomerular Diseases
- Immunology
Background:
- IgA nephropathy (IgAN) and Henoch-Schönlein purpura nephritis (HSPN) are common pediatric glomerular disorders.
- These conditions can lead to end-stage renal disease (ESRD).
- Understanding their pathogenesis is crucial for diagnosis and prognosis.
Purpose of the Study:
- To review the pathogenesis of IgAN and HSPN.
- To discuss the development of diagnostic tests and prognostic markers.
- To present data on long-term outcomes and treatment efficacy.
Main Methods:
- Review of current literature on IgAN and HSPN pathogenesis.
- Analysis of studies on diagnostic and prognostic biomarkers.
- Evaluation of data from randomized controlled trials (RCTs) and clinical outcomes.
Main Results:
- Defective galactosylation of O-linked glycans in IgA1 is implicated in IgAN and HSPN pathogenesis.
- Galactose-deficient IgA1 (Gd-IgA1) in serum shows potential as a diagnostic tool.
- Proteomic techniques offer promise for developing diagnostic and prognostic biomarkers.
- RCTs did not support immunosuppressants for pediatric IgAN/HSPN, but ACE inhibitors are indicated for proteinuria reduction.
Conclusions:
- Childhood IgAN and HSPN carry risks of significant morbidity in childhood and adulthood.
- Future clinical tests will enable noninvasive diagnosis and treatment response monitoring.
- Identifying high-risk individuals is key for preventing progression to ESRD.
Purpose Of Review:
IgA nephropathy and Henoch-Schönlein purpura nephritis are common glomerular disorders in pediatrics that can potentially progress to end-stage renal disease in some patients. This review summarizes our current understanding of the pathogenesis of these closely related conditions and discusses the rationale for development of diagnostic tests and prognostic markers. The review also presents the best data for long-term outcome, clinical markers of prognosis, and the results of randomized controlled trials.
Recent Findings:
Our understanding of the defective galactosylation of O-linked glycans in the hinge region of human IgA1 and its role in the pathogenesis of IgA nephropathy and Henoch-Schönlein purpura nephritis has evolved over the past decade. This review discusses studies that suggest that demonstration of galactose-deficient IgA1 in the serum may become an important diagnostic tool for these conditions. Proteomic techniques for development of biomarkers for diagnosis and prognosis show promise. Although data from randomized controlled trials have failed to support the use of immunosuppressive agents in pediatric IgA nephropathy and Henoch-Schönlein purpura nephritis, recent data indicate that angiotensin converting enzyme inhibitor therapy is indicated for reduction of proteinuria.
Summary:
Childhood IgA nephropathy and Henoch-Schönlein purpura nephritis have the potential for serious morbidity, either during childhood or later in adulthood. In the future clinical tests will be used for noninvasive diagnosis and as markers for judging response to treatment, particularly in those individuals at highest risk for eventual progression to end-stage renal disease.
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