A 23-yr-old white woman presents with recurrent episodes of macroscopic hematuria in the previous 1 yr
By DR. RAM - ABIM BOARD CERTIFIED NEPHROLOGY | Jul 5, 2020
A 23-yr-old white woman presents with recurrent episodes of macroscopic hematuria in the previous 1 yr. Her blood pressure (BP) is 150/95 mmHg. No edema is present. A urinalysis reveals 50 to 100 erythrocytes per high-power field (50% dysmorphic), several erythrocyte casts, and 4 proteinuria. Six months ago, her serum creatinine was 0.9 mg/dl. Her serum creatinine is now 1.9 mg/dl, and a random urine specimen contains 2 g of protein per gram of creatinine. A renal biopsy reveals that 30% of the glomeruli are involved with focal and segmental or circumferential cellular crescents. The remaining glomeruli show mesangial proliferation and focal and segmental glomerulosclerosis. The immunofluorescence study shows 3 IgA, 2 IgG, 1 IgM, 3 C3, negative C1q, and 3 fibrin/fibrinogen.
In addition to blood pressure (BP) control with an angiotensin converting enzyme (ACE) inhibitor, which ONE of the following therapies would you add to her regimen as initial therapy?
Explanation:
This young woman has severe IgA nephropathy with renal insufficiency, moderate proteinuria and a proliferative and crescentic glomerulonephritis. An aggressive approach to treatment is indicated. At the present time, controlled studies support the use of combinations of cyclophosphamide and prednisone as preferred initial therapy in patients with progressive IgA Nephropathy. Oral prednisone alone may be ineffective in patients with already reduced renal function. Fish-oils might be effective, but the presence of crescents points to the need for a more aggressive approach. Cyclosporin has not been shown to be effective in IgA nephropathy and may be harmful. Too few studies have been conducted using mycophenolate mofetil to recommend it as ‘First-line” therapy at the present time.
