Immunotactoid gloemrulonephritis is a glomerular disease characterized by organized microtubular deposits of monoclonal immunoglobulin. These deposits are Congo red-negative, have hollow centers, measure > 30 nm, a...Immunotactoid gloemrulonephritis is a glomerular disease characterized by organized microtubular deposits of monoclonal immunoglobulin. These deposits are Congo red-negative, have hollow centers, measure > 30 nm, and are arranged in stacked or parallel arrays. Treatment of immunotactoid glomerulonephritis is not well-defined, with poor outcomes seen in native kidneys. In fewer than 10% of the cases, trials with steroids alone or cytotoxic agents with steroids or plasmapheresis with steroids has been associated with clinical remission of proteinuria. 50% of patients with renal impairment progress to end stage renal disease (ESRD) in two to four years. There are three case reports of recurrent ITG in renal allograft;one was treated with pulse steroids plus cyclophosphamide, pulse steroids in second case and use of rituximab in the third case. Recurrence of ITG has been described in the renal allograft but there is no literature on relapse of ITG in the native kidneys. Here, we have a case of ITG that relapsed in the native kidneys eight years after being in remission. Renal function improved with fludarabine both times and the patient had stable renal function at last follow-up.展开更多
文摘Immunotactoid gloemrulonephritis is a glomerular disease characterized by organized microtubular deposits of monoclonal immunoglobulin. These deposits are Congo red-negative, have hollow centers, measure > 30 nm, and are arranged in stacked or parallel arrays. Treatment of immunotactoid glomerulonephritis is not well-defined, with poor outcomes seen in native kidneys. In fewer than 10% of the cases, trials with steroids alone or cytotoxic agents with steroids or plasmapheresis with steroids has been associated with clinical remission of proteinuria. 50% of patients with renal impairment progress to end stage renal disease (ESRD) in two to four years. There are three case reports of recurrent ITG in renal allograft;one was treated with pulse steroids plus cyclophosphamide, pulse steroids in second case and use of rituximab in the third case. Recurrence of ITG has been described in the renal allograft but there is no literature on relapse of ITG in the native kidneys. Here, we have a case of ITG that relapsed in the native kidneys eight years after being in remission. Renal function improved with fludarabine both times and the patient had stable renal function at last follow-up.
