| 초록 |
Primary Membranous nephropathy is one of the commonest causes of nephrotic syndrome in the adult population worldwide. Over the last 12 years, significant advances have been made in the diagnosis, monitoring and treatment of patients with membranous nephropathy. This started with the discovery of phospholipase A2 receptor (PLA2R) as a target antigen in about 70-80% of patients with PMN. The ability to stain for this antigen in the kidney biopsy and measure circulating antibodies against it has revolutionised our approach to these patients. A number of other target antigens have been identified in the PLA2R-ve cases and more are being discovered on a regular basis. We can now confidently make a diagnosis of membranes nephropathy by demonstrating the presence of antibodies to PLA2R in blood without the need for a biopsy. Also, the levels are helpful for risk stratification and monitoring of treatment response, since the anti-PLA2R trajectory precedes and is correlated with clinical response. This has prompted the KDIGO glomerular disease guideline working group to recommend shortening of the ‘wait and watch’ period before starting immunosuppressive therapy for cases deemed to be at a high risk of progression. The other area in which there have been significant advances is in treatment of this condition – with a number of clinical trials showing that rituximab, the anti-CD20 monoclonal antibody, is able to induce clinical and serological remission in a substantial proportion of patients. Even though long-term outcomes of rituximab therapy are as yet unknown, this presents an attractive option for many patients to the current standard of care which is a combination of corticosteroids and alkylating agent-based therapies and is associated with a high adverse-effect burden. New drugs are in the pipeline and global clinical trials are being planned to evaluate the effectiveness of these agents. |