Document Detail


Treatment of severe pemphigus with protein A immunoadsorption, rituximab and intravenous immunoglobulins.
MedLine Citation:
PMID:  18070210     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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
Publication Detail:
Type:  Journal Article     Date:  2007-12-06
Journal Detail:
Title:  The British journal of dermatology     Volume:  158     ISSN:  0007-0963     ISO Abbreviation:  Br. J. Dermatol.     Publication Date:  2008 Feb 
Date Detail:
Created Date:  2008-01-24     Completed Date:  2008-04-24     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0004041     Medline TA:  Br J Dermatol     Country:  England    
Other Details:
Languages:  eng     Pagination:  382-8     Citation Subset:  IM    
Affiliation:
Department of Dermatology, University of Lübeck, Ratzeburger Allee 160, 23538 Lübeck, Germany. shim92@hotmail.com
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MeSH Terms
Descriptor/Qualifier:
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
Chemical
Reg. No./Substance:
0/Antibodies, Monoclonal; 0/Immunoglobulins, Intravenous; 0/Immunologic Factors; 0/Staphylococcal Protein A; 0/rituximab

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