- Focal segmental glomerulosclerosis (FSGS) is usually characterized by hypertension, hematuria, renal insufficiency, and nephrotic syndrome.
- Although commonly idiopathic, there are well-described associated mutations in familial cases. FSGS may also be a secondary glomerular disease resulting from decreased nephron mass, obesity, or various other injuries.
- The disease frequently progresses to CKD and ESRD within 5-10 years of diagnosis.
- Pathologic findings include focal and segmental sclerosis of glomeruli on light microscopy with focal or diffuse foot process effacement under electron microscopy.
- It is generally not proven to be effective in the treatment of this disorder, but a trial of prednisone, 60 mg PO daily for at least 3 months, may be appropriate in an effort to reduce proteinuria and slow progression to ESRD in idiopathic FSGS.
- Resistant cases may respond to a combination of glucocorticoids and cytotoxic agents such as cyclosporine, 5 mg/kg/d PO, cyclophosphamide, 2 mg/kg/d PO, or mycophenolate mofetil 1,000-3,000 mg/d. These agents may also reduce the need for steroids in steroid-dependent cases.