title: A 6-year treatment experience for pemphigus: retrospective study of 69 Chinese patients.
abstract: There is a lack of data on treatment and prognosis of pemphigus in China. The aim of this study was to evaluate long-term follow-up and prognosis of pemphigus. Forty-seven inpatients with pemphigus vulgaris (PV) and 22 with pemphigus foliaceus (PF) were recruited in this retrospective study. The average age at onset was 51.6 and 54.9 years in PV and PF, respectively. High-dose systemic steroids were administered in 47 PV and 21 PF, of which 18 PV and 8 PF with adjuvant therapies. CD4 lymphocytopenia was found in 5 PV and 2 PF patients at admission and successfully treated by intravenous thymopentin daily. During a mean follow-up of 37.1 months, 41 PV and 19 PF reached remission, 30 PV and 9 PF relapsed, 4 PV and 2 PF died. Major causes of death were relapse of pemphigus due to discontinuation of oral steroids by the patients themselves (four cases) and severe infections (two cases, one with severe CD4 lymphocytopenia). The 1-year mortality rate of PV and PF was 8.5% and 4.5%, respectively. Cox regression analysis indicated that age at onset of pemphigus was an independent risk factor related to the elevated mortality. Our report confirmed the high mortality rate of pemphigus in a Chinese population and stressed that patient education was urgently needed to prevent relapses and deaths.
authors.affiliation: Department of Dermatology, No.1 Hospital of China Medical University, 155 North Nanjing Street, Shenyang, 110001, China.
authors.forename: Yi-Xiu
authors.initials: YX
authors.lastname: Bai
authors.affiliation: Department of Dermatology, No.1 Hospital of China Medical University, 155 North Nanjing Street, Shenyang, 110001, China.
authors.forename: Li-Ming
authors.initials: LM
authors.lastname: Zhang
authors.affiliation: Department of Dermatology, No.1 Hospital of China Medical University, 155 North Nanjing Street, Shenyang, 110001, China.
authors.forename: Ting
authors.initials: T
authors.lastname: Xiao
authors.affiliation: Department of Dermatology, No.1 Hospital of China Medical University, 155 North Nanjing Street, Shenyang, 110001, China.
authors.forename: Hong-Duo
authors.initials: HD
authors.lastname: Chen
mesh_terms: D000328:Adult; D017668:Age of Onset; D000368:Aged; D002681:China; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000008:administration & dosage / Q000627:therapeutic use*; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D010353:Patient Education as Topic; D010392:Pemphigus / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology; D011379:Prognosis; D016016:Proportional Hazards Models; D012008:Recurrence; D012074:Remission Induction; D012189:Retrospective Studies; D018344:T-Lymphocytopenia, Idiopathic CD4-Positive / Q000188:drug therapy; D016305:Thymopentin / Q000008:administration & dosage / Q000627:therapeutic use*
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D016305:Thymopentin
keywords: CD4 lymphocytopenia; pemphigus; pemphigus foliaceus; pemphigus vulgaris; prognosis; treatment