Abstract
Primary membranous nephropathy (MN) is a frequent cause of NS in adults. In native kidneys the disease may progress to ESRD in the long term, in some 40–50% of untreated patients. The identification of the pathogenic role of anti-podocyte autoantibodies and the development of new therapeutic options has achieved an amelioration in the prognosis of this disease. MN may also develop in renal allograft as a recurrent or a de novo disease. Since the de novo MN may have some different pathogenetic and morphologic features compared to recurrent MN, in the present paper we will deal only with the recurrent disease. The true incidence of the recurrent form is difficult to assess. This is mainly due to the variable graft biopsy policies in kidney transplantation, among the different transplant centers. Anti-phospholipase A2 receptor (PLA2R) autoantibodies are detected in 70–80% of patients. The knowledge of anti-PLA2R status before transplant is useful in predicting the risk of recurrence. In addition, the serial survey of the anti-PLA2R titers is important to assess the rate of disease progression and the response to treatment. Currently, there are no established guidelines for prevention and treatment of recurrent MN. Symptomatic therapy may help to reduce the signs and symptoms related to the nephrotic syndrome. Anecdotal cases of response to cyclical therapy with steroids and cyclophosphamide have been published. Promising results have been reported with rituximab in both prophylaxis and treatment of recurrence. However, these results are based on observational data, and prospective controlled trials are still missing.
Highlights
Membranous nephropathy (MN) is a podocytopathy histologically characterized by uniform thickening of the glomerular basement membrane due to the presence of sub-epithelial immune deposits
It can be secondary to a variety of causes, such as infections, autoimmune diseases, solid tumors, and medical reactions [1, 2] but in most cases it is an autoimmune kidneyspecific disease defined as primary MN, that will be, together with its recurrence after kidney transplantation, the topic of this review
Primary MN is one of the most common causes of nephrotic syndrome (NS) in adults and may lead to end-stage renal disease (ESRD) in the long term in some 40–50% of untreated patients [3,4,5], for whom kidney transplantation is the treatment of choice
Summary
Membranous nephropathy (MN) is a podocytopathy histologically characterized by uniform thickening of the glomerular basement membrane due to the presence of sub-epithelial immune deposits. It can be secondary to a variety of causes, such as infections, autoimmune diseases, solid tumors, and medical reactions [1, 2] but in most cases (about 75%) it is an autoimmune kidneyspecific disease defined as primary MN, that will be, together with its recurrence after kidney transplantation, the topic of this review. Primary MN is one of the most common causes of nephrotic syndrome (NS) in adults and may lead to end-stage renal disease (ESRD) in the long term in some 40–50% of untreated patients [3,4,5], for whom kidney transplantation is the treatment of choice. After transplantation primary MN may develop again, either as a recurrence of the original disease or as a de novo form. We review the diagnostic approach, preventive measures and treatment strategies available for patients with recurrent MN
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