title: Assessment of the rate of long-term complete remission off therapy in patients with pemphigus treated with different regimens including medium- and high-dose corticosteroids.
abstract: BACKGROUND
  Few studies have evaluated pemphigus treatments according to the definitions of the consensus statement. Prognostic factors for complete remission off therapy (CRoffT) remain unknown.
  
  
  OBJECTIVE
  We sought to assess the rate of CRoffT in patients with pemphigus treated with different regimens.
  
  
  METHODS
  In all, 134 patients with pemphigus were included in a retrospective, multicenter study. Primary end point was the rate of CRoffT. Prognostic factors for CRoffT were determined using univariate and multivariate analyses.
  
  
  RESULTS
  Eighty patients with pemphigus vulgaris, 47 with pemphigus foliaceus, and 7 with paraneoplastic pemphigus were included. Mean age was 60 ± 18 years. Patients were treated either with medium (≤0.5 mg/kg/d) (n = 32) or high (≥1 mg/kg/d) (n = 59) doses of prednisone, or without systemic corticosteroids (n = 43). Mean follow-up was 77 ± 64 months. In all, 68 patients (50.7%) achieved CRoffT (95% confidence interval 42.3%-59.2%) after a mean treatment duration of 36 ± 39 months, including 47 of 80 patients with pemphigus vulgaris (58.7%) and 21 of 47 with pemphigus foliaceus (44.7%). Main prognostic factors for CRoffT were initial mucosal involvement (hazard ratio 2.2; 95% confidence interval 1.05-4.58; P = .036) and younger age (<61 years) (hazard ratio 2.5; 95% confidence interval 1.18-5.12; P = .0167). The rate of long-lasting CRoffT was 44%, with a mean follow-up after treatment withdrawal of 59 ± 50 months.
  
  
  CONCLUSIONS
  This was a retrospective study.
  
  
  CONCLUSIONS
  The rate of CRoffT was 51%. Patients with pemphigus vulgaris were more likely to achieve CRoffT than those with pemphigus foliaceus.
authors.affiliation: Department of Dermatology, INSERM U 905, Institute for Research and Innovation in Biomedicine, Rouen University Hospital, University of Rouen, Rouen, France.
authors.forename: Naif
authors.initials: N
authors.lastname: Almugairen
authors.forename: Valerie
authors.initials: V
authors.lastname: Hospital
authors.forename: Christophe
authors.initials: C
authors.lastname: Bedane
authors.forename: Sophie
authors.initials: S
authors.lastname: Duvert-Lehembre
authors.forename: Damien
authors.initials: D
authors.lastname: Picard
authors.forename: Anne-Fleur
authors.initials: AF
authors.lastname: Tronquoy
authors.forename: Estelle
authors.initials: E
authors.lastname: Houivet
authors.forename: Michel
authors.initials: M
authors.lastname: D'incan
authors.forename: Pascal
authors.initials: P
authors.lastname: Joly
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000328:Adult; D000368:Aged; D015331:Cohort Studies; D018450:Disease Progression; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D053208:Kaplan-Meier Estimate; D016015:Logistic Models; D008134:Long-Term Care; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D009173:Mycophenolic Acid / Q000031:analogs & derivatives* / Q000627:therapeutic use; D010392:Pemphigus / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology*; D011237:Predictive Value of Tests; D011241:Prednisone / Q000627:therapeutic use*; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D015996:Survival Rate; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D009173:Mycophenolic Acid; D011241:Prednisone
keywords: CI; CR; CRoffT; CS; MMF; PF; PNP; PV; complete remission; complete remission off therapy; confidence interval; corticosteroids; follow-up; mycophenolate mofetil; paraneoplastic pemphigus; pemphigus; pemphigus foliaceus; pemphigus vulgaris; treatment