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


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.


CONCLUSIONS
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.
Shimanovich
I
I
Department of Dermatology, University of Lübeck, Ratzeburger Allee 160, 23538 Lübeck, Germany. shim92@hotmail.com
Nitschke
M
M
Rose
C
C
Grabbe
J
J
Zillikens
D
D
D000328:Adult; D000368:Aged; D000911:Antibodies, Monoclonal / Q000008:administration & dosage*; D058846:Antibodies, Monoclonal, Murine-Derived; D003131:Combined Modality Therapy / Q000379:methods; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage*; D007155:Immunologic Factors / Q000008:administration & dosage*; D007163:Immunosorbent Techniques; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000188:drug therapy / Q000628:therapy*; D000069283:Rituximab; D013205:Staphylococcal Protein A / Q000008:administration & dosage*; D016896:Treatment Outcome
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D013205:Staphylococcal Protein A; D000069283:Rituximab