Primary membranous nephropathy: comprehensive review and historical perspective

Area of Science:

• Nephrology
• Immunology
• Pathology

Background:

• Membranous nephropathy (MN) is the leading cause of nephrotic syndrome in non-diabetic adults over 40.
• Primary MN affects 8-10 per million individuals, with significant rates of persistent nephrotic syndrome and progression to end-stage renal disease.
• Recent decades have illuminated the pathogenesis and treatment of MN, moving beyond its initial clinic-pathological description.

Purpose of the Study:

• To review recent advancements in understanding the pathogenesis of membranous nephropathy.
• To highlight the significance of novel serological markers and emerging therapeutic options for primary MN.

Main Methods:

• Review of recent literature on membranous nephropathy pathogenesis and treatment.
• Focus on the discovery and implications of anti-PLA2R and anti-THSD7A antibodies.
• Evaluation of newer therapeutic agents like rituximab and adrenocorticotropic hormone analogues.

Main Results:

• Identification of M-type phospholipase A2 receptor (PLA2R) and thrombospondin type 1 domain-containing 7A (THSD7A) antibodies has revolutionized understanding of MN pathogenesis.
• Anti-PLA2R antibody serves as a crucial serological marker for disease prognostication.
• Rituximab and adrenocorticotropic hormone analogues represent promising new therapeutic strategies for primary MN.

Conclusions:

• The discovery of specific antibodies has significantly advanced the understanding of primary membranous nephropathy.
• Anti-PLA2R antibody testing is valuable for predicting disease course.
• Emerging therapies, including rituximab and ACTH analogues, offer new hope for managing primary MN.