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

2007; Oxford University Press; Volume: 158; Issue: 2 Linguagem: Inglês

10.1111/j.1365-2133.2007.08358.x

1365-2133

Iakov Shimanovich, Martin Nitschke, Christian Rosé, Jürgen Grabbe, Detlef Zillikens,

Urticaria and Related Conditions

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