Treatment of primary focal segmental glomerulosclerosis

Abstract

Urinalysis had 4 protein with 5 red blood cells/high-power field along with granular and waxy casts. A 24-hour urine collection contained 8.2 g of protein, and the creatinine clearance was 42 mL/min. The sedimentation rate was 64 mm/h; rheumatoid factor and ANA were normal; C3, 135 mg/dL; C4, 41 mg/dL; and both urine and serum immunoelectrophoresis were negative for paraproteins. A rapid plasma reagin test (RPR) was nonreactive. Hepatitis B surface antigen was negative as was an ELISA evaluation for HIV antibody. A chest radiograph demonstrated her heart to be normal in size, and her lungs were clear, but bilateral pleural effusions were noted. An ultrasound of the kidneys demonstrated them to be normal in size and echogenicity. The renal arteries and veins were patent. A sodiumand water-restricted diet was prescribed, and the patient was treated with furosemide with good response. She underwent a percutaneous renal biopsy 3 weeks after presenCASE PRESENTATION tation. At the time of the biopsy, her blood urea nitrogen was A 53-year-old African American woman who had previously 29 mg/dL; serum creatinine, 3.2 mg/dL; serum albumin, 1.1 been in excellent health presented 11 years ago with increasing g/dL; and cholesterol, 784 mg/dL. weight, leg edema, and facial swelling that had developed over The renal biopsy revealed 17 glomeruli by light microscopy; one month. She had no history of sickle cell disease, recurrent one was hyalinized. The remaining glomeruli manifested mildly urinary tract infections or pyelonephritis, vesicoureteral reflux, increased mesangial matrix and cellularity. The glomerular basediabetes mellitus, hypertension, renal or systemic diseases, or ment membranes were thin and delicate, and the capillary loops morbid obesity. There was no family history of hypertension were patent. One glomerulus had a segmental scar with hyalinoor renal disease. She had not been taking any prescribed medisis, and a second glomerulus had a segmental scar with a small cations nor had she been using nonsteroidal anti-inflammaadhesion to Bowman’s capsule. Acute tubular necrosis and intertory agents. stitial edema were evident but no significant tubulointerstitial Physical examination revealed: blood pressure, 120/84 mm Hg; atrophy or fibrosis was seen. Immunofluorescence examination no skin rashes; lungs were clear; cardiac examination was norof the tissue was negative. Electron microscopy disclosed difmal; no hepatosplenomegaly; and no significant lymphadenopfuse foot process fusion with villous transformation; no elecathy. Her joints were unremarkable but she had 3 pre-tibial tron-dense deposits were seen in the mesangium or along the edema bilaterally. Laboratory evaluation demonstrated a blood capillary wall. urea nitrogen of 32 mg/dL; serum creatinine, 1.8 mg/dL; albuThe patient was given oral prednisone, 60 mg/day; 2 weeks min, 1.6 g/dL; and cholesterol, 612 mg/dL. Hemoglobin was 14.6 later her serum creatinine had fallen to 0.9 mg/dL but the proteing/dL with a normal white blood cell count and platelet count. uria had risen to 12.4 g/day. However, after 2 months of highdose steroid therapy, the proteinuria had decreased to 0.1 g/day, the serum albumin level was 3.8 g/dL, and the cholesterol level Portions of this article are republished with permission from Korbet was down to 326 mg/dL; she was edema free. A steroid taper SM: Primary focal segmental glomerulosclerosis, in Therapy in Nephrolwas completed over an additional 2 months. One week after ogy and Hypertension: A Companion to Brenner and Rector’s The Kidney (2nd ed), edited by Brady RJ, Wilcox CS, Philadelphia, W.B. Saunders discontinuing steroids she had a relapse of the nephrotic syn(in press). drome (13.6 g/day) but again responded to a second course of high-dose prednisone with a complete remission. She remained The Nephrology Forum is funded in part by grants from Amgen, in a complete remission for 4 months after the discontinuation Incorporated; Merck & Co., Incorporated; Dialysis Clinic, Incorpoof steroids and then relapsed a second time (proteinuria of 13 rated; and Bristol-Myers Squibb Company. g/day). She was treated with prednisone and oral cyclophospha