HLA-DR7 predicts the response to alkylating agents in steroid-sensitive nephrotic syndrome

• Published in: Pediatric nephrology (Berlin, West) West), 1997-02, Vol.11 (1), p.16-19
• Main Authors: KONRAD, M, MYTILINEOS, J, RUDER, H, OPELZ, G, SCHÄRER, K
• Format: Article
• Language: English
• Subjects: Adolescent; Age of Onset; Alkylating Agents - therapeutic use; Anti-Inflammatory Agents - therapeutic use; Biological and medical sciences; Child; Child, Preschool; Chlorambucil - therapeutic use; Cyclophosphamide - therapeutic use; DNA - analysis; Female; Glomerulonephritis; HLA-DR7 Antigen - blood; Humans; Infant; Male; Medical sciences; Nephrology. Urinary tract diseases; Nephropathies. Renovascular diseases. Renal failure; Nephrotic Syndrome - blood; Nephrotic Syndrome - drug therapy; Polymorphism, Restriction Fragment Length; Prednisone - therapeutic use; Retrospective Studies; Steroids - therapeutic use
• ISSN: 0931-041X; 1432-198X
• DOI: 10.1007/s004670050224

There is a lack of reliable predictors of the response to alkylating agents in children with idiopathic nephrotic syndrome (NS). HLA-DR7 is strongly associated with the frequency of relapses in steroid-sensitive NS before cytostatic therapy. We therefore examined retrospectively the time to the first relapse and the incidence of subsequent relapses in 54 HLA-typed children with frequently relapsing NS, after treatment with cyclophosphamide (n = 49) or chlorambucil (n = 5) for 8 or 12 weeks; 38 patients were HLA-DR7 positive and 16 negative with 80% in both groups being steroid dependent. HLA typing was performed using serological or DNA typing methods. Renal biopsy showed minimal glomerular changes. A lower proportion of HLA-DR7 positive than negative patients remained in remission after 3 years (36% vs. 81%, P < 0.02) and 5 years (36% vs. 72%, P < 0.03). In the first 3 years after cytostatic therapy the mean number of prednisone-treated relapses was 1.3/patient per year in HLA-DR7-positive patients compared with 0.4 in negative patients (P < 0.025). There was no statistically significant difference in the proportion of relapse-free patients with and without steroid dependency. The HLA status predicts the response of NS patients to alkylating agents better than the rate of previous relapses.