Successful treatment with rituximab of immunotactoid glomerulopathy exhibiting nephrotic syndrome

As immunotactoid glomerulopathy (ITG) is a very rare primary glomerular disease, no standard treatment has been established. It has been reported that ITG progresses to end-stage renal disease at a high rate. Here, we report a case of ITG exhibiting nephrotic syndrome treated by administration of a single dose of rituximab every 6 months for 4 years. In this case, complete remission (CR) was not achieved with steroids alone, but was achieved through long-term depletion of B cells by administration of rituximab. This is the first report that single-dose rituximab every 6 months for 4 years not only achieved CR of ITG, but also allowed steroid tapering.