| 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. |
| Authors | I Shimanovich, M Nitschke, C Rose, J Grabbe, D Zillikens
(Affiliation: Department of Dermatology, University of Lübeck, Ratzeburger Allee 160, 23538 Lübeck, Germany. shim92 at hotmail.com)
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| Journal | The British journal of dermatology
(Br J Dermatol)
Vol. 158
Issue 2
Pg. 382-8
(Feb 2008)
ISSN: 0007-0963 England |
| PMID | 18070210
(Publication Type: Journal Article)
|
| Chemical References |
- Antibodies, Monoclonal
- Immunoglobulins, Intravenous
- Immunologic Factors
- Staphylococcal Protein A
- rituximab
|
| Topics |
- Adult
- Aged
- Antibodies, Monoclonal
(administration & dosage)
- 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, therapy)
- Staphylococcal Protein A
(administration & dosage)
- Treatment Outcome
|
