Treatment of severe pemphigus with protein A immunoadsorption, rituximab and intravenous immunoglobulins

Br J Dermatol. 2008 Feb;158(2):382-8. doi: 10.1111/j.1365-2133.2007.08358.x. Epub 2007 Dec 6.

Abstract

Background: Pemphigus is a life-threatening autoimmune blistering disease usually treated with high-dose corticosteroids and other immunosuppressants. However, this regimen may prove inadequate in severe cases and cause dangerous side-effects. While protein A immunoadsorption (PAIA) induces a rapid remission in severe pemphigus, the disease usually recurs once the treatment is stopped. In contrast, anti-CD20 antibody rituximab has a delayed onset of action but may lead to a long-term remission of pemphigus.

Objective: To develop a treatment protocol combining the rapid remission induced by PAIA with the positive long-term effects of rituximab.

Patients and methods: Five patients with pemphigus vulgaris and two patients with pemphigus foliaceus were treated with a combination of PAIA, rituximab and conventional immunosuppressants. Patients who failed to respond to this therapy subsequently received intravenous immunoglobulins (IVIg).

Results: All seven patients showed a sharp decline of circulating autoantibody levels and rapid improvement of cutaneous and mucosal lesions within 4 weeks of therapy. Long-term remission was induced in three patients and one further patient showed a partial improvement of his disease. The three remaining patients who could not be weaned off PAIA and remained resistant to rituximab treatment showed a good response to IVIg therapy.

Conclusion: The combination of PAIA and rituximab induces a rapid and durable remission in a subset of patients with severe pemphigus. IVIg therapy appears to be a good treatment option for rituximab nonresponders.

MeSH terms

  • Adult
  • Aged
  • Antibodies, Monoclonal / administration & dosage*
  • Antibodies, Monoclonal, Murine-Derived
  • Combined Modality Therapy / methods
  • Drug Therapy, Combination
  • Female
  • Humans
  • Immunoglobulins, Intravenous / administration & dosage*
  • Immunologic Factors / administration & dosage*
  • Immunosorbent Techniques*
  • Male
  • Middle Aged
  • Pemphigus / drug therapy
  • Pemphigus / therapy*
  • Rituximab
  • Staphylococcal Protein A / administration & dosage*
  • Treatment Outcome

Substances

  • Antibodies, Monoclonal
  • Antibodies, Monoclonal, Murine-Derived
  • Immunoglobulins, Intravenous
  • Immunologic Factors
  • Staphylococcal Protein A
  • Rituximab