Fb. Waldo et al., THERAPY OF FOCAL AND SEGMENTAL GLOMERULOSCLEROSIS WITH METHYLPREDNISOLONE, CYCLOSPORINE-A, AND PREDNISONE, Pediatric nephrology, 12(5), 1998, pp. 397-400
Patients with steroid-resistant focal and segmental glomerulosclerosis
(FSGS) have a poor prognosis but may benefit from high-dose methylpre
dnisolone or cyclosporine A therapy. Ten patients were treated with a
protocol of methylprednisolone infusions for 8 weeks followed by a com
bination of cyclosporine A and alternate-day prednisone for maintenanc
e of remission for 2 weeks. Eight of ten patients remitted the nephrot
ic syndrome within 8 weeks of beginning treatment. One patient remitte
d edema but remained proteinuric, and one did not respond. After obser
vation for 12-24 months, seven patients maintained remission with norm
al glomerular filtration rate. One non-responder had renal insufficien
cy and one patient had secondary non-response and end-stage renal dise
ase. No patients developed hypertension. One patient had the diagnosis
of Hodgkin disease made after 10 months of therapy. Follow-up renal b
iopsy in four patients showed no evidence of progressive interstitial
disease, There were no other major side effects. Steroid-resistant FSG
S may be successfully treated with the described protocol. Additional
studies will be needed to determine if this approach prevents progress
ion of renal disease.