Our understanding of the pathogenesis of primary MN has advanced greatly with the identification of M-type
phospholipase A2 receptor and
thrombospondin type-1 domain-containing 7A as target
antigens whose
antibodies serve as
biomarkers of this disease. Additional research, including investigations into the roles of
complement and
melanocortin receptors on the podocyte, may further improve our understanding of how best to treat this condition. Immunosuppressive therapies, including
corticosteroids alternating with
alkylating agents, and
calcineurin inhibitors are partially successful in reducing
proteinuria in MN, but their use may be associated with significant adverse effects and a high relapse rate. Novel interventions, including targeting B cells with
rituximab as well as treatment with
adrenocorticotropic hormone (
ACTH), are being investigated. Key Messages: The understanding of treatment targets and availability of new
biomarkers has facilitated diagnosis and improved risk stratification for MN and may also be useful for individualizing treatment with a wider range of therapeutic options for patients with MN. Considerable evidence supports the use of B-cell depletion as initial
therapy in nephrotic patients with MN.
ACTH should be considered for patients who do not respond to traditional
therapies such as
alkylating agents and
calcineurin inhibitors.