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

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 m(2). Patients in the RIM group were more likely to have lower eGFR (100.8 mL/min/1.73 m(2) vs 132.9 mL/min/1.73 m(2), 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.