, Volume 27, Issue 5, pp 813-819
Date: 25 Nov 2011

Renin–angiotensin–aldosterone system inhibitors in pediatric focal segmental glomerulosclerosis

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Conventional immunosuppressive therapy for primary pediatric focal segmental glomerulosclerosis (FSGS) is potentially toxic and only moderate evidence supports its effectiveness. Renin–angiotensin–aldosterone (RAAS) inhibition monotherapy is anecdotally used in selected patients as an alternative to conventional therapy.


We performed a retrospective cohort study of children with primary FSGS seen at a tertiary care academic hospital between 1986 and 2008. We classified patients into two groups based upon initial treatment: RAAS inhibition monotherapy (RIM) and conventional therapy (CT). The primary endpoint was progression to end-stage renal disease (ESRD). Secondary endpoints were remission of proteinuria, relapse, and death.


The cohort consisted of 67 patients. Mean baseline urine protein/creatinine ratio (Up/c) was 8.0 (5.2, 10.7) mg/mg, and mean baseline estimated glomerular filtration rate (eGFR) was 115.0 (101.8, 128.1) mL/min/1.73 m2. Patients in the RIM group were more likely to have lower eGFR (100.8 mL/min/1.73 m2 vs 132.9 mL/min/1.73 m2, p = 0.01) and less proteinuria (4.4 vs.14.4, p < 0.01). Renal failure occurred in 22.9% of the RIM group vs 40.6% in the CT group (log-rank p = 0.07). After adjustment for African–American race, time period of presentation, baseline age, eGFR, and Up/c, patients in the RIM group had a 0.11 hazard ratio of progressing to renal failure compared with patients in the CT group (p < 0.01).


Children treated initially with RIM may have better outcomes than those treated with CT.