Molecular Dissection of Target Antigens and Nephritogenic Antibodies in Membranous Nephropathy: Towards Epitope-Driven Therapies

[摘要] Membranous nephropathy (MN) is the most common cause of idiopathic nephrotic syndrome in white adults, accounting for about 20% of cases. The disease is characterized by an accumulation of immune deposits on the outer aspect of the glomerular basement membrane (GBM). The immune deposits consist of IgG (often IgG4), thus far unidentified antigens, and the membrane attack complex of complement C5b-9. Although spontaneous remission of nephrotic syndrome occurs in about a third of patients, MN ends for about 40% of patients in end-stage renal failure after 10 yr (1). Treatment of MN is often disappointing (2,3). This is due in part to heterogeneity of the disease and lack of reliable biomarkers because of ignorance of the target antigen(s) and nephritogenic antibodies. Strategies to target B-lymphocytes with anti-CD20 antibody (4) and to inhibit complement (5) are steps in the right direction, but more specific, concept-driven therapies are urgently needed.

[学科分类] 泌尿医学

[关键词] Bone marrow necrosis;Sickle cell disease;Hyperhemolysis syndrome