Therapy-resistant focal and segmental glomerulosclerosis.
The clinical course of a young female patient with focal segmental glomerulosclerosis (FSGS) who failed to respond to any of the recommended therapeutic protocols will represent the background of a discussion of currently available alternative treatments for FSGS. Traditionally, FSGS has been believed to have a poor prognosis, with a low response rate to treatment and a progressive course terminating with end-stage renal disease (ESRD). Some 40% of patients respond to prolonged corticosteroid treatment. Steroid resistance in adults should perhaps be assumed only after failure to respond to a 6-month course of daily steroid therapy. Regarding recent recommendations, the use of cytotoxic therapy (cyclophosphamide, chlorambucil or azathioprine) may be considered as second-line therapy (evidence D). Treatment with cyclosporin A at doses of 4-6 mg/kg/day has been successful in reducing proteinuria. There is little information available on the effects of such treatment on the progression of FSGS. Even fewer data are available on the success rate of the use of tacrolimus in resistant forms of FSGS in adults. Mycophenolate mofetil has been used with impressive success in a few high-risk patients who failed on previous therapeutic regimens. There is preliminary evidence in an uncontrolled series of patients with resistant primary FSGS that the addition of plasmapheresis may provide effective long-term benefits in some patients. The accurate assessment of the role of plasmapheresis and possibly immunoadsorption in the management of patients with FSGS requires further evaluation. Non-immunosuppressive therapy (i.e. angiotensin-converting enzyme inhibitors, angiotensin II receptor blockers, lipid-lowering drugs, non-steroidal anti-inflammatory drugs) should be applied to almost all patients with primary FSGS.
Kveder
Radoslav
R
Department of Nephrology, University Medical Center, Ljubljana, Slovenia. radoslav.kveder@mf.uni-lj.si
D000293:Adolescent; D001707:Biopsy, Needle; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D005923:Glomerulosclerosis, Focal Segmental / Q000150:complications / Q000473:pathology* / Q000628:therapy*; D006801:Humans; D007150:Immunohistochemistry; D007677:Kidney Function Tests; D008775:Methylprednisolone / Q000008:administration & dosage; D009404:Nephrotic Syndrome / Q000209:etiology / Q000473:pathology* / Q000628:therapy*; D010956:Plasmapheresis / Q000379:methods*; D018570:Risk Assessment; D017211:Treatment Failure
D003520:Cyclophosphamide; D008775:Methylprednisolone