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Treatment of severe Henoch-Schönlein and immunoglobulin A nephritis. A single center experience
Stella Edström Halling1, Magnus P Söderberg, Ulla B Berg
1Department of Clinical Science, Intervention and Technology, Division of Pediatrics, Karolinska Institutet, Karolinska University Hospital, Huddinge, 14186, Stockholm, Sweden. stella.edstrom.halling@ki.se
Insights
Two treatment regimens for severe pediatric nephritis showed similar outcomes. Both corticosteroid, cyclophosphamide, and ACEi/ARB therapy, and ACEi/ARB +/- corticosteroids effectively reduced proteinuria and improved kidney function in Henoch-Schönlein nephritis and IgA nephritis.
Area of Science:
- Nephrology
- Pediatric Nephrology
- Immunology
Background:
- Severe Henoch-Schönlein nephritis (HSN) and immunoglobulin A nephritis (IgAN) in children can lead to significant kidney damage.
- Treatment strategies for severe pediatric nephritis vary, necessitating research into their comparative efficacy.
Purpose of the Study:
- To compare the effectiveness of two distinct treatment regimens in children with severe HSN and IgAN.
- To evaluate the impact of treatments on renal function, proteinuria, and histological findings.
Main Methods:
- A cohort of 43 children with severe HSN (24) and IgAN (19) were analyzed.
- Group A received corticosteroids, cyclophosphamide (CYC-P), and ACE inhibitors/angiotensin receptor blockers (ACEi/ARB).
- Group B received ACEi/ARB with or without corticosteroids.
Main Results:
- Both treatment groups showed decreased proteinuria at follow-up.
- Group A demonstrated increased GFR and a reduced histopathological activity index.
- Treatment outcomes were comparable between the two groups and did not differ between HSN and IgAN.
Conclusions:
- Combined therapy with corticosteroids, CYC-P, and ACEi/ARB is effective in improving renal parameters in severe pediatric nephritis.
- ACEi/ARB therapy, with or without corticosteroids, also shows benefits in reducing proteinuria.
- Treatment efficacy did not significantly differ between the two regimens or between HSN and IgAN.
Abstract:
Our aim was to report the effect of two treatment regimens in 43 cases of severe Henoch-Schönlein nephritis (HSN) and immunoglobulin A nephritis (IgAN) (24 HSN, 19 IgAN). Group A, 11 HSN and 7 IgAN, 88% with an International Study of Kidney Disease in Children (ISKDC) biopsy grade > or = III and severe clinical features, were treated with corticosteroids, cyclophosphamide (CYC-P) and angiotensin-converting enzyme inhibitor/angiotensin receptor blocker (ACEi/ARB). Group B, 12 HSN and 13 IgAN, 72% with biopsy findings as above and 52% with severe clinical features, were treated with ACEi/ARB +/- corticosteroids. The outcome classification was: (a) healthy; (b) mild proteinuria, normal glomerular filtration rate (GFR); (c) active renal disease; (d) chronic renal failure. Twenty-six patients had a good outcome (a + b). The 17 children with poor outcome (c + d) had lower GFR at onset and at follow-up, higher albumin excretion at follow-up, and higher percentage of segmental glomerulosclerosis in the renal biopsy, than those with good outcome. Treatment with corticosteroids, CYC-P and ACEi/ARB was effective in increasing GFR, reducing proteinuria and decreasing the disease activity index. The proteinuria had decreased at follow-up in both groups. In group A, GFR increased and histopathological activity index declined after treatment. The outcome did not differ between groups A and B. The effects of treatment did not differ between HSN and IgAN.
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