What is the role of Plasmapheresis (PLEX) for vasculitis?

From the Guidelines

Plasmapheresis (PLEX) is recommended for patients with ANCA-associated vasculitis (AAV) and severe manifestations, such as diffuse alveolar hemorrhage or rapidly progressive glomerulonephritis, as it can rapidly remove circulating antibodies and provide immediate relief. The decision to use PLEX should be individualized, taking into account the severity of the disease and the patient's overall condition. According to the most recent guidelines, PLEX is recommended for AAV patients with serum creatinine levels ≥500 μmol/L due to rapidly progressive glomerulonephritis or severe diffuse pulmonary hemorrhage 1. The typical regimen involves 5-7 exchanges over 7-14 days, with each session removing approximately 1-1.5 plasma volumes. PLEX is usually administered alongside immunosuppressive therapy, such as cyclophosphamide and high-dose corticosteroids, to rapidly remove circulating antibodies and suppress new antibody production. Some key points to consider when using PLEX include:

• Monitoring for potential complications, such as bleeding, hypocalcemia, and infection risk
• Close monitoring of coagulation parameters, calcium levels, and signs of infection during treatment
• Individualizing the decision to use PLEX based on the severity of the disease and the patient's overall condition
• Using PLEX in combination with immunosuppressive therapy to achieve optimal outcomes The PEXIVAS trial, a recent randomized controlled trial, demonstrated that a reduced dose regimen of glucocorticoid can reduce serious infections at 1 year compared with the standard dose regimen group 1. However, the trial failed to show a reduction in the composite outcome of death from any cause or end-stage kidney disease in patients with severe AAV randomized to plasma exchange in addition to immunosuppressive therapy compared with immunosuppressive therapy alone. Overall, PLEX can be a valuable treatment option for patients with AAV and severe manifestations, but its use should be carefully considered and individualized based on the patient's specific condition and needs.

From the Research

Role of Plasmapheresis (PLEX) in Vasculitis

• Plasmapheresis (PLEX) has been used to treat severe renal vasculitis and/or alveolar haemorrhage in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) 2.
• The use of PLEX in AAV is based on its ability to rapidly remove ANCA IgG, complement, and coagulation factors important in the pathogenesis of AAV 3.
• PLEX has been shown to be effective in patients with severe renal involvement, particularly those with creatinine levels >500 μmol/L or dialysis-dependent 3, 4.
• It is also considered in patients with creatinine levels >300 μmol/L and rapidly deteriorating function, or with life-threatening pulmonary hemorrhage 3.
• A separate indication for PLEX is in patients double positive for anti-GBM antibodies and ANCA 3.

Indications and Outcomes

• PLEX is often used in combination with immunosuppressive agents to achieve early disease control and prevent resynthesis of ANCA 3.
• Studies have shown that PLEX can improve renal function and reduce the risk of end-stage kidney disease in patients with AAV 2, 4.
• However, the PEXIVAS trial did not show a benefit of adjunctive PLEX on a combined endpoint of end-stage kidney disease and death 3, 5.
• Observational studies have reported a recovery of alveolar hemorrhage following extracorporeal treatment, including PLEX 5, 4.

Safety and Adverse Events

• Adverse events attributable to PLEX have been reported in up to 63% of patients, but no deaths occurred during PLEX 4.
• The most common indications for PLEX are life-threatening or organ function-threatening manifestations, particularly advanced renal dysfunction and disease refractory to traditional therapy 6.
• Randomized controlled studies are needed to define optimal indications, PLEX regimen, and concomitant medications 6, 4.