Document Detail


Long-term prognosis of pemphigus in Korea: retrospective analysis of 199 patients.
MedLine Citation:
PMID:  22025028     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: Pemphigus is the most common and severe autoimmune bullous disease in Korea.
OBJECTIVE: The aim of this clinical study was to evaluate the clinical features, treatment modalities and long-term prognosis of pemphigus in Korea.
METHODS: We conducted a retrospective analysis of 199 patients diagnosed with pemphigus vulgaris (PV) and pemphigus foliaceus (PF) between 1993 and 2008. Complete and partial remissions were evaluated according to the definitions proposed by the International Pemphigus Committee.
RESULTS: The mean age of onset was 46.1 years, and the female-to-male ratio was 1.1:1.0 (women: 102, men: 97). The ratio of PV to PF was 1.1:1.0 (PV: 104, PF: 95). Complete/partial remission rate for PV was 77% at 5 years and 94% at 10 years after initial diagnosis. The corresponding rate for PF was 87% at 5 years and 98% at 10 years after initial diagnosis. There was no difference in time to remission between mild cases (treated with prednisolone, Pd, alone) and severe cases (treated with Pd ± adjuvant therapy). Sixteen patients, who were recalcitrant to conventional therapy, received rituximab; all of these achieved overall remission without adverse effects. Eight (8%) PV patients and 5 (5%) PF patients died during the 16-year follow-up period.
CONCLUSIONS: Most patients reached remission within 10 years of treatment. Pd + adjuvant therapy did not yield a better prognosis than Pd alone. All patients who received rituximab experienced remarkable clinical improvement. Rituximab is an effective and safe drug for patients who are resistant to conventional therapy.
Authors:
Mi Ri Kim; Hyeon Chang Kim; Soo-Chan Kim
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Publication Detail:
Type:  Journal Article     Date:  2011-10-21
Journal Detail:
Title:  Dermatology (Basel, Switzerland)     Volume:  223     ISSN:  1421-9832     ISO Abbreviation:  Dermatology (Basel)     Publication Date:  2011  
Date Detail:
Created Date:  2011-11-29     Completed Date:  2012-04-18     Revised Date:  2013-05-29    
Medline Journal Info:
Nlm Unique ID:  9203244     Medline TA:  Dermatology     Country:  Switzerland    
Other Details:
Languages:  eng     Pagination:  182-8     Citation Subset:  IM    
Copyright Information:
Copyright © 2011 S. Karger AG, Basel.
Affiliation:
Department of Dermatology and Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea.
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MeSH Terms
Descriptor/Qualifier:
Adult
Aged
Anti-Infective Agents / therapeutic use*
Anti-Inflammatory Agents / therapeutic use*
Antibodies, Monoclonal, Murine-Derived / therapeutic use
Azathioprine / therapeutic use
Cyclophosphamide / therapeutic use
Cyclosporine / therapeutic use
Dapsone / therapeutic use
Drug Therapy, Combination
Female
Humans
Immunoglobulins, Intravenous / therapeutic use
Immunologic Factors / therapeutic use*
Immunosuppressive Agents / therapeutic use*
Male
Methylprednisolone / therapeutic use
Middle Aged
Mycophenolic Acid / analogs & derivatives,  therapeutic use
Pemphigus / drug therapy*,  pathology
Prednisolone / therapeutic use*
Prognosis
Republic of Korea
Retrospective Studies
Severity of Illness Index
Time Factors
Treatment Outcome
Young Adult
Chemical
Reg. No./Substance:
0/Anti-Infective Agents; 0/Anti-Inflammatory Agents; 0/Antibodies, Monoclonal, Murine-Derived; 0/Immunoglobulins, Intravenous; 0/Immunologic Factors; 0/Immunosuppressive Agents; 0/rituximab; 24280-93-1/Mycophenolic Acid; 446-86-6/Azathioprine; 50-18-0/Cyclophosphamide; 50-24-8/Prednisolone; 59865-13-3/Cyclosporine; 80-08-0/Dapsone; 83-43-2/Methylprednisolone; 9242ECW6R0/mycophenolate mofetil

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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