Treatment of severe Henoch–Schönlein and immunoglobulin A nephritis. A single center experience

• Published in: Pediatric nephrology (Berlin, West) Vol. 24; no. 1; pp. 91 - 97
• Main Authors: Edström Halling, Stella, Söderberg, Magnus P., Berg, Ulla B.
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 2009; Springer; Springer Nature B.V
• Subjects: Adolescent; Age of Onset; Angiotensin II Type 1 Receptor Blockers - therapeutic use; Angiotensin-Converting Enzyme Inhibitors - therapeutic use; Biopsy; Child; Child, Preschool; Children & youth; Cyclophosphamide - therapeutic use; Drug Therapy, Combination; Enzymes; Female; Glomerular Filtration Rate; Glomerulonephritis, IGA - drug therapy; Glomerulonephritis, IGA - pathology; Glomerulonephritis, IGA - physiopathology; Hematuria; Hospitals; Humans; Immunoglobulins; Kidney diseases; Longitudinal Studies; Male; Medicin och hälsovetenskap; Medicine & Public Health; Methylprednisolone - therapeutic use; Nephrology; Original Article; Pediatrics; Proteinuria - drug therapy; Purpura, Schoenlein-Henoch - drug therapy; Purpura, Schoenlein-Henoch - pathology; Purpura, Schoenlein-Henoch - physiopathology; Retrospective Studies; Steroids; Treatment Outcome; Urology; Sweden; Cyclophosphamide; Renal biopsy findings; IgA nephritis; Segmental glomerulosclerosis; Henoch–Schönlein nephritis; Methylprednisolone; Proteinuria; Glomerular filtration rate
• ISSN: 0931-041X; 1432-198X
• DOI: 10.1007/s00467-008-0990-z

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 ≥ 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.