Membranous nephropathy
Membranous nephropathy is the leading cause of nephrotic syndrome in white adults, most often a primary autoimmune disease driven by anti-PLA2R antibodies against a podocyte antigen, diagnosed by kidney biopsy or noninvasively by anti-PLA2R serology. Patients with persistent nephrotic-range proteinuria after a period of conservative observation are treated with cyclical glucocorticoid-cyclophosphamide, a calcineurin inhibitor, or rituximab.
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What this article covers
- Overview
The leading cause of nephrotic syndrome in white adults: membranous nephropathy (also known as membranous glomerulopathy) has a peak incidence between 30 and 50 years of age, with epidemiology that has remained constant over recent decades.
- Epidemiology
Most common cause of the nephrotic syndrome in White adults, with a stable incidence over the past several decades and a peak between 30 and 50 years of age.
- Risk factors
Age 30-65 years, the peak incidence window for primary disease; age 65 and older carries an elevated risk that the process is malignancy-associated.
- Pathophysiology
Circulating autoantibodies target a podocyte antigen: in most primary cases, autoantibodies against the M-type phospholipase A2 receptor (PLA2R) permeate the glomerular basement membrane and form…
- Clinical features
Slowly progressive edema is the presenting complaint in most patients, developing over weeks as proteinuria accumulates.
- Differential diagnosis
Minimal change disease: nephrotic syndrome with a normal glomerulus on light microscopy and effacement of podocyte foot processes only on electron microscopy, without the subepithelial deposits and…
- Diagnosis
The diagnosis is traditionally made by kidney biopsy, which shows subepithelial immune deposits altering the glomerular capillary wall, classically with "spikes" of glomerular basement membrane…
- Management
First, observe on conservative therapy for 6 to 12 months in every newly diagnosed patient with primary membranous nephropathy, since this allows a chance for spontaneous remission, which occurs in…
- Complications
Venous and arterial thromboembolism: the most distinctive complication, including renal vein thrombosis, occurring in up to 25% of patients and most likely within the first 2 years; risk rises…
- Prognosis
Progression to ESKD depends directly on remission status: the 10-year incidence ranges from 0% to 10% with complete or partial remission, compared with more than 50% with persistent nephrotic-range proteinuria.
- Prevention
No primary prevention exists for idiopathic (anti-PLA2R-mediated) disease, since the autoantibody trigger is not modifiable.
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