Effective treatment of rapidly deteriorated renal function with methylprednisolone pulse therapy and high dose mycophenolate mofetil in A patient with lupus nephritis

A 30 year old African American female with Graves' disease and unspecified connective tissue disorder was admitted because of orthopnea and general edema. Laboratory testing showed blood urea nitrogen levels of 60 mg/dL, creatinine of 4.6 mg/dL, serum albumin of 2.1 g/dL and the daily urinary protein loss was 4.6 g. C3 level was 78 mg/dL and C4 level was 36 mg/dL. The antinuclear antibody titer was 1:640, with a speckled pattern, and anti-ds DNA was positive. She was negative for lupus anticoagulant. Renal biopsy revealed focal segmental proliferative lupus nephritis, and immunofluorescent microscopy showed full house immune deposits and electron microscopy revealed diffusely enlarged podocytes with foot process effacement and characteristic tubuloreticular inclusions in endothelial cells. She received 1 g/day of pulse methylprednisolone intravenously for 3 days, followed by 60 mg/day of prednisone. She was also started with mycophenolate mofetil 1000mg twice a day and followed by decreased dosage. Heavy proteinuria and serum creatinine level started improving after 5 days of treatment and nephrotic syndrome remitted completely in 3 weeks. The differential diagnosis, clinical risk factors and manifestations, prognostic predictors and treatment are discussed.