Nephrology

“Rapid removal of antibodies is crucial in patients at high risk of renal function loss due to high antibody titers and severe symptoms associated with immunological disorders”.

Nephrology

Membranous Nephropathy

Membranous glomerulonephritis (MGN) is an organ-specific autoimmune disease and represents the leading cause of nephrotic syndrome in adults. Formation of sub-epithelial immune deposits and complement activation lead to alterations in the structure of the glomerular basement membrane and damage to the glomerular filtration barrier, causing proteinuria. The natural history of idiopathic MGN presents with three main clinical courses: spontaneous remission occurs in 30% of cases, while persistent proteinuria is observed in the remaining 70%, with half progressing to end-stage renal disease (ESRD).

In this condition, phospholipase A2 receptor 1 (PLA2R) represents the main podocyte antigen involved in the pathogenesis of idiopathic MGN in adults. Furthermore, a link has been established between serum levels of anti-PLA2R antibodies and the clinical course of the disease (proteinuria levels, response to therapy).

Currently, standard treatment involves corticosteroids, immunosuppressants, and monoclonal antibodies. However, there are cases where pharmacological therapy alone is insufficient to counteract high antibody titers. In these instances, patients require targeted plasma clearance of antibodies involved in pathogenesis through Double Filtration Plasmapheresis and Cascade Filtration.

Molecules to be removed: Anti-PLA2R antibodies
Recommended Therapies: Plasma Exchange (PEX), Double filtration plasmapheresis (DFPP), Cascade Filtration (CF)

Goodpasture syndrome is an autoimmune disorder characterized by alveolar hemorrhage and glomerulonephritis, caused by circulating anti-glomerular basement membrane (anti-GBM) antibodies. Symptoms include dyspnea, cough, asthenia, hemoptysis, and hematuria, and diagnosis is confirmed by the presence of anti-GBM antibodies in blood or renal biopsy samples. Treatment includes plasma exchange, corticosteroids, and immunosuppressants.

Molecules to be removed: Anti-glomerular basement membrane antibodies (anti-GBM), IgG
Recommended Therapies: Plasma Exchange (PEX), Double filtration plasmapheresis (DFPP), Cascade Filtration (CF)

Post-infectious glomerulonephritis is a specific type of renal glomerular inflammation that can compromise its filtering capacity. It is often caused by nephritogenic strains of group A β-hemolytic streptococcus. Microbial antigens bind to the glomerular basement membrane and activate the complement pathway through interaction with circulating antibodies, resulting in glomerular damage. Symptoms can range from asymptomatic hematuria and mild proteinuria to nephritis with micro or macrohematuria, proteinuria, oliguria, edema, hypertension, and renal failure. Treatment options include plasma exchange, aimed at removing involved circulating antibodies.

Molecules to be removed: Immune complexes
Recommended Therapies: Plasma Exchange (PEX), Double filtration plasmapheresis (DFPP), Cascade Filtration (CF)

Rapidly progressive glomerulonephritis is a condition characterized by extensive formation of glomerular crescents that, if untreated, progresses to end-stage kidney disease within weeks or months. The condition presents in four distinct patterns:

  • Rapidly progressive glomerulonephritis due to immune complexes (type 2), complicating numerous infectious and connective tissue disorders, and occurring with other primary glomerulopathies. Immunofluorescence shows nonspecific granular immune deposits. This disease accounts for up to 40% of cases of rapidly progressive glomerulonephritis, and its pathogenesis is typically unknown
  • Dual antibody disease (type 4) presents with antibodies such as anti-MBG and ANCA.
  • Idiopathic rapidly progressive glomerulonephritis affects patients with immune complexes, connective tissue diseases, or glomerulopathies
  • Absence of ANCA is known as type V rapidly progressive glomerulonephritis. Treatment involves corticosteroids and plasma exchange (PEX), with the aim of removing circulating antibodies involved.

Molecules to be eliminated: Autoantibodies
Recommended Therapies: Plasma Exchange (PEX), Double filtration plasmapheresis (DFPP), Cascade Filtration (CF)

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