Plasmapheresis for systemic vasculitis.

Area of Science:

• Rheumatology and Nephrology
• Immunology
• Internal Medicine

Background:

• Systemic vasculitides encompass diverse diseases, with the 2012 Chapel Hill Consensus Conference (CHCC2012) providing a current classification framework.
• Plasmapheresis (PEX) is utilized for various vasculitides, but systematic reviews correlating its efficacy with the CHCC2012 classification are limited.

Purpose of the Study:

• To review the efficacy and latest evidence of plasmapheresis for systemic vasculitides, categorized by the CHCC2012 classification.
• To focus on small vessel vasculitides and the role of PEX in their treatment.

Main Methods:

• Systematic review of existing literature and clinical trial data.
• Analysis of plasmapheresis efficacy based on the CHCC2012 vasculitis classification.
• Examination of evidence for small vessel vasculitides, including ANCA-related vasculitis and anti-GBM nephritis.

Main Results:

• Plasmapheresis demonstrates accumulated efficacy data for immune complex vasculitis, with strong recommendations for anti-GBM nephritis.
• Recent randomized controlled trials (RCTs), like the PEXIVAS trial, show no significant reduction in mortality or renal death for ANCA-related vasculitis (AAV).
• Current guidelines classify PEX as salvage or selective therapy for severe AAV cases, despite negative RCT findings.

Conclusions:

• Plasmapheresis efficacy varies across vasculitis types; it is strongly recommended for anti-GBM nephritis but questioned in AAV.
• The complex nature of vasculitis and combination therapies make isolated PEX efficacy assessment challenging.
• Further research is needed to identify potential treatment-sensitive subgroups that may benefit from plasmapheresis.