Clinical And Prognostic Analysis Of 96 Patients With Pemphigus

Objective:To review the clinical data and explore factors affecting prognosis of patients with pemphigus.Methods:The clinical data of inpatients with pemphigus vulgaris（PV）and pemphigus foliaceus（PF）who had been treated in Department of Dermatology,The First Hospital of China Medical University from 2015 to 2021 were retrospectively analyzed,and the clinical manifestations,laboratory examination,treatment and prognosis of the patients were analyzed.The Kaplan-Meier method and Log-rank test was used for survival univariate analysis.The Cox stepwise regression model was used for multivariate analysis.Absence of new or established lesions,while receiving predniso≤10 mg/day and/or minimal adjuvant therapy,or off all systemic therapy,for at least 2 months,were defined as complete remission（CR）on therapy（CRon T）and CR off therapy（CRoff T）,respectively.Results:A total of 96 patients with pemphigus were enrolled,including 67 patients with PV and 29 patients with PF.Among 67 patients with PV,the ratio of male to female was 1:1.39,the age of onset was 57.21±14.54（19～87）years.Among 29patients with PF,the ratio of male to female was 1.42:1,the age of onset was 62.24±14.15（21～88）years.For a mean period of 38.55±22.10 months,29（43.28%）and 8（11.94%）patients with PV reached CRon T and CRoff T,13（44.83%）and 10（34.48%）patients with PF reached CRon T and CRoff T,respectively.Univariate analysis showed that the adverse factors for CRoff T were age of onset≥50 years(?_Log-Rank²=4.49,P=0.034)and disease duration≤6 months(?_Log-Rank²=5.32,P=0.021)in patients with PV and patients with PF,respectively.Cox regression analysis showed that PV subtype（HR 0.257,95%CI 0.096～0.688,P=0.007）and disease duration≤6 months（HR 0.287,95%CI 0.109～0.756,P=0.012）were negatively correlated with CRoff T.A total of 12 patients with PV died,and the mean age of death was73.92±10.30（56～88）years.Three were over 70 years old and four were over 80years old.The 1-year,2-year and 5-year mortality rates were 10.4%,16.4%and17.9%,respectively.Three patients with PF died and the ages of death were 94,84and 85 years old,respectively.Conclusion:The age of onset≥50 years and the disease duration≤6 months are adverse factors of reaching CRoff T in patients with PV and patients with PF,respectively.Patients with PF reached CRoff T more quickly than patients with PV and patients with PF had a higher rate of CRoff T than patients with PV.The mortality rate of patients with PV remains high and is dominated by older patients.