| 초록 |
FSGS is a common primary glomerular histologic lesion associated with high-grade proteinuria and with ESRD.
Primary FSGS is defined by exclusion of any other identifiable cause of secondary FSGS. Most patients with persistent
nephritic range proteinuria are progress, but patients with non-nephrotic proteinuria are at low risk for progressive
kidney failure and ESRD.
The first treatment approach consists of optimal boold pressure control and the use of renin-angiotensin system
inhibitors, statins, a low-salt diet, and diuretic. The treatment goal of FSGS is to induce a complete remission of proteinuria
that in turn will lead to better long-term preservation of renal function. Achieving partial remission, although not
optimal, does slow the progression of kidney disease and substantially improve renal survival. The current KDIGO guideline
on GN recommends initial treatment of primary FSGS with high-dose prednisone given for between 4 and 16 weeks
or until complete remission. CNIs are recommended for patients with FSGS who are resistant or intolerant to glucocorticoids
and are continued for a minimum of 1 year if the patient is responsive. There is insufficient evidence to support
the use of alkylating agents, MMF, rituximab or apheresis in the treatment of FSGS, these therapy may have a role in
patients who are resistant or intolerant to conventional treatment.
Recently, Circulating soluble urokinase receptor (suPAR) was found as a circulating permeability factor in primary
FSGS. Further studies are needed to validate the therapeutic importance of this biomarker in patients with primary
FSGS. |