Alternative Therapies to Immunoglobulin for Autoimmune Blistering Diseases

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Project Status:
Completed
Project Line:
Health Technology Review
Project Sub Line:
Summary with Critical Appraisal
Project Number:
RC1483-000

Question

  1. What is the clinical effectiveness of alternative treatments to IVIg compared to IVIg or placebo for autoimmune blistering diseases?
  2. What is the safety of alternative treatments to IVIg compared to IVIg or placebo for autoimmune blistering diseases?
  3. What are the evidence-based guidelines regarding the use of alternative treatments to IVIg for autoimmune blistering diseases?

Key Message

  • We did not find any evidence regarding the clinical effectiveness and safety of alternative treatments to IV immunoglobulin (IVIg) compared to IVIg or placebo for bullous pemphigoid (BP) or pemphigus vulgaris (PV) and pemphigus foliaceus (PF) that met our inclusion criteria for this review.
  • We identified 6 consensus guidelines presenting treatment algorithms for BP (3 guidelines) or PV and PF (3 guidelines). All guidelines recommend that IVIg may be used as a third-line treatment for severe or refractory cases.
  • For severe or refractory BP, other therapeutic options than IVIg include monoclonal antibodies, immunosuppressive drugs, immunoadsorption, and plasma exchange (3 guidelines).
  • For severe or refractory PV and PF, other therapeutic options than IVIg include immunosuppressive drugs, dapsone, immunoadsorption, plasma exchange, and IV corticosteroid pulse therapy (3 guidelines).
  • The evidence base supporting these guidelines was unclear; recommendations should be interpreted with caution.