Table 5.
Setting | Therapy | Comment |
---|---|---|
Nephrotic forms of primary FSGS,a APOL1 FSGS, certain steroid-sensitive genetic forms of FSGS | Prednisone, initially daily or alternate daysa | Alternate for patients at high risk for steroid complications: calcineurin inhibitorsa |
Steroid-resistant FSGS with nephrotic syndromea | Calcineurin inhibitora (cyclosporin and possibly, tacrolimus) | |
Refractory FSGS with nephrotic syndromea | Mycophenolate mofetil plus high-dose dexamethasonea | |
All forms of FSGS with subnephrotic proteinuria | ACE inhibitor and angiotensin receptor blocker; dietary sodium restriction | Thiazide diuretic may potentiate the antiproteinuric of RAAS antagonism |
Therapy doses are in ref. 46. Cyclosporin has been shown effective in randomized, controlled trials, whereas tacrolimus has not. In the nosology presented here, these recommendations would apply, when nephrotic syndrome is present, to primary FSGS, APOL1 FSGS, and certain rare forms of genetic FSGS that may be steroid sensitive. ACE, angiotensin-converting enzyme; RAAS, renin-angiotensin-aldosterone system.
Recommendations from the Kidney Disease Improving Outcomes Global initiatives for idiopathic FSGS with nephrotic syndrome are extended here to other forms of FSGS as shown.