Spontaneous Remission Of Therapy-resistant Minimal Change Nephritis In An Adult Woman 12 Years After Onset Of The Disease

Abstract

A 23-year old woman was admitted to our hospital because of severe edema due to steroid resistant minimal change nephritis (MCN). The diagnosis was proven by renal biopsy nine years ago. At that time, steroid therapy led to a complete remission. Seven years later, patient was 22 years old, a relapse with severe nephrotic syndrome occurred. The diagnosis MCN was confirmed by a second renal biopsy. A combined therapy with prednisolone and cyclosporine A (CSA) led only to a partial reduction of protein excretion, the edema did not disappear. After 3 months, patient declined further therapy with CSA. On admission to our hospital, one year later in December 2000, the woman showed a severe nephrotic syndrome with edema and fluid lung, despite high doses of furosemide. Urinary protein excretion was 12.5 g/day, serum creatinine was increased to 1.4 mg/dl, the serum protein was reduced to 47 g/l. A repeated renal biopsy confirmed again the diagnosis MCN. Once again, a steroid bolus monotherapy over 4 weeks and an immunosuppressive therapy with CSA over 6 weeks had no effect on proteinuria. Further therapy regimes with mofetil mycophenolat, azathioprine, chlorambucil and cyclophosphamide over a period of 6-12 weeks of each regime was not well tolerated, proteinuria remained high with > 10 g/day. Moreover the patient suffered from severe edema despite furosemide infusions. Therefore, an additional mechanical ultrafiltration was performed 2-4 times monthly. Three months after the last immunosuppressive therapy the edema disappeared spontaneously, the diuretic therapy could be stopped. Serum creatinine was 0.8 mg/dl, protein in urine was still high with 9.8 g/day but serum protein for the first time was normal with 65 g/l. Three months later, the protein excretion was reduced to 0.48 g/l, and all other laboratory data were normal. Meanwhile, the woman has now enjoyed a complete second spontaneous remission for a period of three years.