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Henoch-Schönlein purpura nephritis in children: risk factors, prevention and treatment
1The Institute of Mother and Child Healthcare of Serbia "Dr Vukan Cupić", Belgrade, Serbia. maloun@eunet.rs
Insights
Older age and severe Henoch-Schönlein purpura (HSP) symptoms increase risk for kidney disease (HSPN). Current evidence does not support prednisone for prevention, and more research is needed for treatment.
Area of Science:
- Pediatric Nephrology
- Rheumatology
- Immunology
Background:
- Henoch-Schönlein purpura (HSP) is a common childhood vasculitis.
- HSP can lead to Henoch-Schönlein purpura nephritis (HSPN), potentially causing long-term renal impairment.
- Identifying risk factors and effective treatments for HSPN is crucial.
Purpose of the Study:
- To identify risk factors for developing HSPN and progressive kidney disease in children with HSP.
- To review current evidence on the efficacy of treatments for preventing and managing established renal disease in HSPN.
- To highlight the need for further research, particularly well-designed randomized controlled trials (RCTs).
Main Methods:
- A comprehensive literature review was conducted.
- Studies published over the last two decades were analyzed.
- Focus was placed on identifying risk factors and evaluating treatment efficacy.
Main Results:
- Older age, persistent/recurrent purpura, and severe abdominal symptoms are significant risk factors for HSPN.
- Children with nephritic/nephrotic syndrome or significant glomerular crescents/lesions at presentation face higher risks of long-term renal impairment.
- Randomized controlled trials (RCTs) do not support short-course prednisone for preventing persistent renal disease in HSP. Data on treatment for established HSPN is limited and lacks strong RCT support.
Conclusions:
- Clinical presentation and histological findings at HSPN onset predict long-term renal outcomes.
- Severity and duration of extrarenal symptoms, along with older age, are key predictors for developing HSPN.
- Well-designed RCTs are essential for evaluating treatments in moderately severe or rapidly progressive HSPN.
Aim:
To identify risk factors for a child with Henoch-Schönlein purpura (HSP) either to develop nephritis (HSPN) or to contract progressive course and to obtain the currently available evidence on the efficacy of treatment options in both preventing and treating the established renal disease.
Method:
Review of the literature published over the last two decades.
Results:
Persistent or recurrent purpura, severe abdominal symptoms and an older age proved as the most significant risk factors for later HSPN. The risks of long-term renal impairment are the highest in children having at presentation nephritic/nephrotic syndrome and/or more than 50% of glomeruli occupied by large crescents or sclerosing lesions. Randomized controlled trials (RCT) do not support short course prednisone at presentation of HSP in preventing persistent renal disease. Many uncontrolled studies using various treatment regimens have reported outcomes considered better than expected. However, the data from RCTs are sparse and no treatment options for the established renal disease can be currently recommended based on RCTs.
Conclusion:
Severity and/or duration of extrarenal HSP symptoms and an older age are the most significant risk factors for developing HSPN, whereas clinical and histological severity at HSPN onset are in general predictive of a long-term renal impairment. The existing evidence does not support of short course prednisone in preventing persistent renal disease. A well-designed RCTs are needed in children with moderately severe or rapidly progressive (crescentic) HSPN.
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