Therapeutic targets in membranous nephropathy: plasma cells and complement.
Clin Kidney J
; 17(9): sfae243, 2024 Sep.
Article
in En
| MEDLINE
| ID: mdl-39239361
ABSTRACT
Membranous nephropathy (MN) is an antibody-mediated autoimmune disease and the most common cause of nephrotic syndrome in adults. The discovery of phospholipase A2 receptor 1 (PLA2R1) as the first target antigen in patients with MN 15 years ago has led to a paradigm shift in the pathobiological understanding of this disease. Autoantibodies against PLA2R1 as well as thrombospondin type-1 domain-containing 7A, the second identified antigen in adults, were shown to be disease-causing and act through local activation of the complement system, primarily via the classical and lectin pathways. These findings indicate that both plasma cells, the main source of antibodies and autoantibodies, as well as the complement system, the main pathogenic effector mechanism in MN, are rational and pathogenesis-based treatment targets in MN. This review summarizes pathomechanistic and clinical evidence for and against plasma cell- and complement-targeted treatments in MN.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Language:
En
Journal:
Clin Kidney J
Year:
2024
Document type:
Article
Affiliation country:
Germany
Country of publication:
United kingdom