Pulse Dexamethasone in Focal Segmental Glomerulosclerosis

Date de début de l'étude : 1 mars 2000

INCLUSION CRITERIA: Adults and children. Biopsy proven FSGS, including idiopathic FSGS and collapsing FSGS, but excluding HIV-associated FSGS and secondary FSGS associated with morbid obesity, sickle cell anemia, reflux nephropathy, chronic tubular injury, congenital renal anomalies, and reduced nephron mass. Glomerular filtration rate will be estimated using 4 variable MDRD GFR equation, which incorporates age, race, gender, and serum creatinine. If disease has been present less than or equal to 1 year, estimated GFR must be greater than or equal to 40 ml/min. If disease has been present greater than or equal to 1 year, estimated GFR must be greater than or equal to 60 ml/min. Nephrotic range proteinuria, defined as urine protein greater than or equal to 3.5 g/1.73 m(2)/d. Patients who received steroids for FSGS and who entered complete remission but have relapsed with nephrotic range proteinuria will be eligible. If hypertensive, adequate blood pressure control (target BP less than or equal to 130/80 mm Hg at greater than 75% of measurement in adults). Patients must have been taking an angiotensin converting enzyme inhibitor or angiotensin receptor antagonist for at least 6 weeks, unless intolerant of these medications. Women with child-bearing potential must maintain an effective birth control regimen (oral contraceptive, intrauterine device, barrier plus spermicide). EXCLUSION CRITERIA: Inability to give informed consent or cooperate with study. Poorly controlled diabetes (as defined by hemoglobin A1C of greater than or equal to 8.5 on entry screening, or daily insulin requirement of greater than or equal to 100 units) or hypertension (defined as systolic BP consistently greater than 160 and/or diastolic BP consistently greater than 100 on three or more drugs). Evidence of chronic or occult infection. Specifically, must not have evidence of active hepatitis B, hepatitis C, HIV or untreated mycobacterial infection. Current or prior use of cytotoxic agents or cyclosporin for FSGS. If previously treated with these agents for a non-renal indication, therapy must have concluded at least 12 months prior to study. Existence of any other condition which would complicate the implementation or interpretation of the study. History of steroid-induced psychiatric disorder, known avascular necrosis of hip or symptomatic osteoporosis (e.g., known compression fractures), brittle diabetes mellitus, or glaucoma. Patients with other psychiatric disorders will be evaluated on a case by case basis. Patients who have been treated with steroids equivalent to 1 mg/kg/day for greater than or equal to 8 weeks without remission will be excluded.