What are the indications for plasma exchange in Systemic Lupus Erythematosus (SLE)?

Indications for Plasma Exchange in Systemic Lupus Erythematosus (SLE)

Plasma exchange in SLE should be reserved for specific life-threatening or refractory conditions, primarily thrombotic thrombocytopenic purpura (TTP), diffuse pulmonary hemorrhage, and catastrophic antiphospholipid syndrome. 1

Primary Indications

1. Thrombotic Microangiopathy (TMA)

• Thrombotic Thrombocytopenic Purpura (TTP)
  • Confirmed by ADAMTS13 activity ≤10% 1
  • Treatment protocol:
    • Immediate plasma exchange in adults with intermediate-to-high PLASMIC score while awaiting ADAMTS13 results
    • Combined with high-dose glucocorticoids
    • May add rituximab and/or caplacizumab (von Willebrand factor inhibitor) 1
  • In children: Consider deferring plasma exchange for 24-48 hours until ADAMTS13 results confirm necessity 1

2. Pulmonary Manifestations

• Diffuse Pulmonary Hemorrhage
  • Add plasmapheresis to standard immunosuppressive therapy 1
  • Used as adjunctive therapy with corticosteroids and immunosuppressants 2, 3

3. Antiphospholipid Syndrome (APS)

• Catastrophic APS
  • Characterized by rapid-onset thrombosis affecting multiple organs 1
  • Treatment includes triple therapy:
    • Therapeutic anticoagulation
    • High-dose glucocorticoids
    • Plasma exchange 1, 4
  • Associated with improved patient survival in retrospective studies 1

4. Overlap Syndromes

• ANCA Vasculitis and Anti-GBM GN Overlap
  • Add plasmapheresis according to anti-GBM GN criteria 1

Secondary Indications (Refractory Cases)

1. Refractory Lupus Nephritis

• Consider in patients who fail to respond to:
  • Standard therapy with cyclophosphamide or mycophenolate mofetil
  • Alternative agents including rituximab, calcineurin inhibitors 1, 2
• Most beneficial in:
  • Rapidly progressive lupus nephritis with high immunological activity 5
  • Acute nephrotic syndrome within 1 year of onset 5
  • Histological activity score >20 5

2. Neuropsychiatric SLE

• Reserved for severe, therapy-resistant manifestations 2, 3
• Used as adjunctive therapy with standard immunosuppression

3. Hematologic Manifestations

• Consider in severe, refractory cases of:
  • Hemolytic anemia (better response compared to thrombocytopenia) 6
  • Hyperviscosity syndrome 2, 3
  • Cryoglobulinemia 2, 3

Implementation Considerations

Treatment Protocol

• Typically administered as:
  • 5-10 sessions (median 6.5 sessions) 6
  • Used in conjunction with corticosteroids and immunosuppressants 2

Monitoring

• Track disease activity using validated tools (e.g., SLEDAI)
  • Significant reductions in SLEDAI scores have been observed after TPE (16.7±8.3 before vs. 8.8±3.1 after) 6

Potential Complications

• Major adverse events may include:
  • Catheter infections
  • Bleeding
  • Hypotension 6

When to Avoid Plasma Exchange

• Chronic renal failure or stable renal function (CCr >50 ml/min) 5
• Chronic nephrotic syndrome >1 year with past history of nephrotic syndrome 5
• Low immunological activity and mild renal histology 5
• Cases where risks outweigh potential benefits

Emerging Technologies

• Immunoadsorption techniques may offer advantages over traditional plasma exchange 2, 3
• Consider in combination with newer biologics in refractory cases 3

Plasma exchange remains a valuable rescue therapy in specific SLE scenarios despite limited evidence from controlled trials. The decision to use plasma exchange should be based on the severity of the manifestation, refractoriness to standard therapy, and the specific SLE sub-phenotype, with particular attention to TTP, diffuse pulmonary hemorrhage, and catastrophic APS where it has shown the most benefit.