title: Prediction of survival for patients with bullous pemphigoid: a prospective study.
abstract: OBJECTIVE
  To identify the prognostic factors of bullous pemphigoid (BP).
  
  
  METHODS
  Prospective study of patients with BP included in a randomized, controlled trial.
  
  
  METHODS
  Twenty dermatology departments in France. Patients One hundred seventy patients with BP initially treated with a 40-g/d dosage of clobetasol propionate cream (testing sample) and 171 patients initially treated with oral corticosteroids at a dosage of 0.5 or of 1.0 mg/kg per day, depending on the extent of BP (validation samples).
  
  
  METHODS
  The end point was overall survival during the first year after BP diagnosis. From the testing sample, associations of clinical and biological variables with overall survival were assessed using univariate and multivariate analyses. Selected predictors were included in a prognostic model. To verify that these predictors were not dependent on the treatment used, the model was then validated independently on the 2 series of BP patients treated with oral corticosteroids.
  
  
  RESULTS
  Median age of the BP patients included in the testing sample was 83 years. The 1-year Kaplan-Meier survival rate was 74%. From univariate analysis, the main deleterious predictors were demographic factors (ie, older age and female sex), associated medical conditions (ie, cardiac insufficiency, history of stroke, and dementia), and low Karnofsky score, which is a measure of the patient's general condition. No factors directly related to BP, in particular extent of cutaneous lesions, were shown to be related to the patients' prognosis. From multivariate analysis, only older age (P = .02) and low Karnofsky score (P<.001) appeared independently predictive of death. From the Cox model including these 2 predictors, the predicted 1-year survival rates were 90% (95% confidence interval [CI], 85%-96%) for patients 83 years or younger with Karnofsky score greater than 40, 79% (95% CI, 69%-90%) for patients older than 83 years with Karnofsky score greater than 40, 65% (95% CI, 50%-86%) for patients 83 years or younger with Karnofsky score of 40 or less, and 38% (95% CI, 26%-57%) for patients older than 83 years with Karnofsky score of 40 or less. Kaplan-Meier survival distributions of patients from the validation samples appeared clearly separated according to these 4 categories and were in close agreement with corresponding predicted 1-year survival rates obtained from the testing sample.
  
  
  CONCLUSIONS
  The prognosis of patients with BP is influenced by age and Karnofsky score. These predictors are easy to use and should facilitate the management of BP.
authors.affiliation: Department of Dermatology, Institut National de la Santé et de la Récherche Médicale U 519, University of Rouen, Rouen, France. Pascal.Joly@chu-rouen.fr
authors.forename: Pascal
authors.initials: P
authors.lastname: Joly
authors.forename: Jacques
authors.initials: J
authors.lastname: Benichou
authors.forename: Catherine
authors.initials: C
authors.lastname: Lok
authors.forename: Marie France
authors.initials: MF
authors.lastname: Hellot
authors.forename: Philippe
authors.initials: P
authors.lastname: Saiag
authors.forename: Emmanuelle
authors.initials: E
authors.lastname: Tancrede-Bohin
authors.forename: Bruno
authors.initials: B
authors.lastname: Sassolas
authors.forename: Bruno
authors.initials: B
authors.lastname: Labeille
authors.forename: Marie Sylvie
authors.initials: MS
authors.lastname: Doutre
authors.forename: Isabelle
authors.initials: I
authors.lastname: Gorin
authors.forename: Christine
authors.initials: C
authors.lastname: Pauwels
authors.forename: Olivier
authors.initials: O
authors.lastname: Chosidow
authors.forename: Frédéric
authors.initials: F
authors.lastname: Caux
authors.forename: Eric
authors.initials: E
authors.lastname: Estève
authors.forename: Yves
authors.initials: Y
authors.lastname: Dutronc
authors.forename: Michèle
authors.initials: M
authors.lastname: Sigal
authors.forename: Catherine
authors.initials: C
authors.lastname: Prost
authors.forename: Hervé
authors.initials: H
authors.lastname: Maillard
authors.forename: Jean Claude
authors.initials: JC
authors.lastname: Guillaume
authors.forename: Jean Claude
authors.initials: JC
authors.lastname: Roujeau
mesh_terms: D000284:Administration, Oral; D000287:Administration, Topical; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000704:Analysis of Variance; D002423:Cause of Death; D002990:Clobetasol / Q000627:therapeutic use*; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D005260:Female; D006801:Humans; D008297:Male; D015999:Multivariate Analysis; D010391:Pemphigoid, Bullous / Q000175:diagnosis* / Q000188:drug therapy* / Q000401:mortality*; D011237:Predictive Value of Tests; D011336:Probability; D011379:Prognosis; D016016:Proportional Hazards Models; D011446:Prospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D002990:Clobetasol