Conflicts of interest None declared
Treatment of severe pemphigus with protein A immunoadsorption, rituximab and intravenous immunoglobulins
Version of Record online: 7 DEC 2007
British Journal of Dermatology
Volume 158, Issue 2, pages 382–388, February 2008
How to Cite
Shimanovich, I., Nitschke, M., Rose, C., Grabbe, J. and Zillikens, D. (2008), Treatment of severe pemphigus with protein A immunoadsorption, rituximab and intravenous immunoglobulins. British Journal of Dermatology, 158: 382–388. doi: 10.1111/j.1365-2133.2007.08358.x
- Issue online: 7 DEC 2007
- Version of Record online: 7 DEC 2007
- Accepted for publication 30 August 2007
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.