Pemphigus in Korea a retrospective analysis of 199 patients over a 16-year period

Abstract

[한글]

[영문]Background: Pemphigus is a group of autoimmune blistering diseases of skin and mucous membrane with severe morbidity and occasional mortality. It is necessary to investigate the clinical features, treatment and long-term outcome of Korean patients with pemphigus as it is the most common and severe autoimmune bullous disease in Korea. Objective: The aim of this study was to evaluate the clinical features, treatment outcomes and long-term prognoses of Korean patients with pemphigus. Methods: We conducted a retrospective analysis of 199 patients diagnosed with pemphigus in Gangnam Severance Hospital between 1993 and 2008. Out of 199 total patients, 104 patients had pemphigus vulgaris (PV), and 95 patients had pemphigus foliaceus (PF). Treatment outcomes were calculated using Kaplan-Meier method and the pre-and post-rituximab severity scores were compared using a paired t test. Results: We found that the mean age of onset was 46.1 ± 14.6 years, and the male to female ratio was equal. Systemic corticosteroids were the mainstay of treatment, and azathioprine was the most frequently used adjuvant therapy. We assessed treatment outcome according to a consensus statement on the definition of the disease proposed by the International Pemphigus Committee. Overall remission (complete plus partial remission) was induced in 21%, 77% and 93% in PV patients 1,5 and 10 years after diagnosis, respectively, and in 51%, 87% in PF patients 1 and 5 years after diagnosis, respectively (p < .001, log rank test). Eight (7%) PV patients and five (5%) PF patients died during 16-year follow-up period. Sixteen patients (PV: 15, PF: 1) who were recalcitrant to conventional therapy received rituximab, and all of these patients achieved overall remission after 3-4 months from initiating treatment with rituximab. Mean pre- and post-rituximab pemphigus severity scores were 12.9 and 2.9 respectively ( p < .001).Conclusion: From this clinical study which followed up 199 Korean patients with pemphigus, most patients with pemphigus eventually could reach remission within 10 years of treatment. We confirmed that rituximab is a very effective and safe drug for severe pemphigus patients who are resistant to conventional therapy with early remissions being achieved.