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Long-term prognosis of pemphigus in Korea: retrospective analysis of 199 patients

Authors
 Kim M.R.  ;  Kim H.C.  ;  Kim S.-C. 
Citation
 DERMATOLOGY, Vol.223(2) : 182-188, 2011 
Journal Title
DERMATOLOGY
ISSN
 1018-8665 
Issue Date
2011
MeSH
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 ; Mycophenolic Acid/therapeutic use ; Pemphigus/drug therapy* ; Pemphigus/pathology ; Prednisolone/therapeutic use* ; Prognosis ; Republic of Korea ; Retrospective Studies ; Rituximab ; Severity of Illness Index ; Time Factors ; Treatment Outcome ; Young Adult
Keywords
Pemphigus vulgaris ; Pemphigus foliaceus ; Korea ; Clinical study ; Prognosis
Abstract
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.
Full Text
http://www.karger.com/Article/FullText/332848
DOI
10.1159/000332848
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Dermatology (피부과학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Preventive Medicine (예방의학교실) > 1. Journal Papers
Yonsei Authors
Kim, Miri(김미리)
Kim, Soo Chan(김수찬) ORCID logo https://orcid.org/0000-0002-2327-4755
Kim, Hyeon Chang(김현창) ORCID logo https://orcid.org/0000-0001-7867-1240
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/94742
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