widespread/global in the remainder. 40 (70.2%) showed evidence of an increase in mesangial matrix. By definition all biopsies showed mesangial electron dense deposits. Five (13.1%) biopsies showed evidence of scanty electron dense deposits in other regions, including sub-endothelial in four (7.0%) and sub-epithelial in one (1.8). Clinical progression Patients were followed up for a median 40 months (range 1 113 months). At the time of renal – biopsy, 22 (38.6%) patients had an eGFR <60 ml/min. 16 (28.1%) patients reached the primary in this study, namely the doubling of serum creatinine from baseline at the time of renal biopsy. By the end of this study, seven (12.3%) patients reached ESRD, of whom five (8.8%) received a renal transplant. There was no evidence of recurrent disease in any post-transplant biopsy. Treatment response All patients received standard therapy with maximum tolerated ACE inhibitors +/- ARBs. Each patient’s nephrologist then selected adjuvant therapy. In total, 22 (38.6%) patients received immunosuppression of any kind, this included prednisolone in 11 (19.3%), tacrolimus in 10 (17.5%), MMF in three (5.3%), rituximab in three (5.3%), and cyclophosphamide in one (1.6%). There was no significant difference in progression to doubling of serum creatinine between those patients treated with any kind of immunosuppression and those who were not. Out of 23 patients presenting with the nephrotic syndrome, 14 (60.9%) achieved PR after mean 160 days (range 27 771), while nine (39.1%) achieved CR after mean 93 days (range 36 226). Renal – – outcome was significantly better (p <0.005) in those who achieved CR compared to those who did not. Two of the nine patients who achieved a complete remission subsequently had a relapse of their nephrotic syndrome. Repeated biopsy
widespread/global in the remainder. 40 (70.2%) showed evidence of an increase in mesangial matrix. By definition all biopsies showed mesangial electron dense deposits. Five (13.1%) biopsies showed evidence of scanty electron dense deposits in other regions, including sub-endothelial in four (7.0%) and sub-epithelial in one (1.8). Clinical progression Patients were followed up for a median 40 months (range 1 – 113 months). At the time of renal biopsy, 22 (38.6%) patients had an eGFR <60 ml/min. 16 (28.1%) patients reached the primary in this study, namely the doubling of serum creatinine from baseline at the time of renal biopsy. By the end of this study, seven (12.3%) patients reached ESRD, of whom five (8.8%) received a renal transplant. There was no evidence of recurrent disease in any post-transplant biopsy. Treatment response All patients received standard therapy with maximum tolerated ACE inhibitors +/- ARBs. Each patient’s nephrologist then selected adjuvant therapy. In total, 22 (38.6%) patients received immunosuppression of any kind, this included prednisolone in 11 (19.3%), tacrolimus in 10 (17.5%), MMF in three (5.3%), rituximab in three (5.3%), and cyclophosphamide in one (1.6%). There was no significant difference in progression to doubling of serum creatinine between those patients treated with any kind of immunosuppression and those who were not. Out of 23 patients presenting with the nephrotic syndrome, 14 (60.9%) achieved PR after mean 160 days (range 27 – 771), while nine (39.1%) achieved CR after mean 93 days (range 36 – 226). Renal outcome was significantly better (p <0.005) in those who achieved CR compared to those who did not. Two of the nine patients who achieved a complete remission subsequently had a relapse of their nephrotic syndrome. Repeated biopsy
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