SUN-408 catastrophic minimal change disease; an unusual presentation of a usual disease

Abstract

Minimal change disease (MCD) is a major cause of idiopathic nephrotic syndrome (NS), characterized by intense proteinuria leading to edema and intravascular volume depletion. In adults, it accounts for approximately 10-15% of patients with idiopathic NS. The pathologic hallmark of disease is absence of visible alterations by light microscopy and effacement of foot processes by electron microscopy. The mainstay of therapy is prednisone, but steroid-sensitive forms frequently relapse and this leads to a percentage of patients requiring second-line steroid-sparing immunosuppression. However, in a substantial number of patients the disease is recurrent and requires long-term immunosuppression, with significant morbidity because of side effects. Recent therapeutic advances, such as the use of anti-CD20 antibodies, have provided long-term remission off-therapy and suggest new hypotheses for disease pathogenesis. our case report is about a case of minimal change (proven by electron microscope) with a very aggressive and progressive course despite intense medical treatment reaching end stage renal disease requiring renal replacement therapy. our case is 27 years old female patient married with 2 children youngest is 5 years old, housewife, no past medical history, no family history of renal disease. patient presented by nephrotic syndrome, renal biopsy was done and steroids was started by 60 mg/day till results of renal biopsy. later on, biopsy showed minimal change disease by electron microscopy but the patient did not show response to steroid therapy after 2 months and showed significant side effects of steroid therapy, a steroid free regimen was started with cyclosporine for another 4 months, protienuria decreased initially but recurred again and more aggressive. we decided to add mycophenolate mofetil to our regimen for better control in attempt to achieve remission. our patient did not show response with persistent nephrotic-range protienuria and serum creatinine started to rise. we decided to shift to endoxan 1gm/month for 6 doses, partial response was obtained and partial remission was maintained for another 6 months. unfortunately, our patient had relapse again with nephrotic range protienuria more than 10 gm/mg and significant edema and progressive rise in renal functions. In addition to best supportive therapy, we decided to start rituximab for 2 doses trying to achieve remission and control of protienuria and aggressive disease process but again no response and the patient reached end stage renal disease and needed renal replacement therapy. At this stage we doubted our first diagnosis and decided to do another renal biopsy to confirm this aggressive pathology. while maintaining the patient on maintenance haemodialysis, the pathologist found 6 0ut of 13 glomeruli with global glomerulosclerosis with 25% interstitial fibrosis and tubular atrophy. It seems not to be always simple to manage minimal change disease in adults as in children, as it carries much higher risk and poorer prognosis.