title: Risk factors for lethal outcome in patients with bullous pemphigoid: low serum albumin level, high dosage of glucocorticosteroids, and old age.
abstract: BACKGROUND
  Although bullous pemphigoid (BP) is the most frequent autoimmune bullous disease and is associated with a considerable case-fatality rate, little is known about factors that influence its prognosis.
  
  
  OBJECTIVE
  To identify prognostic factors for lethal outcome in the first year after the initial hospitalization in patients with BP.
  
  
  METHODS
  A multicenter retrospective cohort study.
  
  
  METHODS
  Seven dermatologic university hospitals in Germany.
  
  
  METHODS
  A total of 369 patients diagnosed as having BP between January 1, 1987, and December 31, 1997.
  
  
  METHODS
  Univariate (Kaplan-Meier) and multivariate (Cox regression) analysis.
  
  
  RESULTS
  Of the 369 patients with BP, 209 (57%) died, 106 (29%) within the first year after hospitalization. Fifty-four percent were women. The mean +/- SD age at entry was 77.3 +/- 11.1 years. The patients with BP were followed up to 10.5 years, with a median time of 1.8 years to death or interview (25th and 75th quartiles, 0.5 and 4.0 years). The major risk factors for lethal outcome in the first year after hospitalization were an increased age, with a multivariate risk estimate of 3.2 (95% confidence interval [CI], 1.9-5.2) for age greater than 80.4 years (median); a daily glucocorticosteroid dosage of more than 37 mg (75th quartile) at discharge, with a multivariate risk estimate of 2.5 (95% CI, 1.5-4.3); serum albumin levels of 3.6 g/dL or less (25th quartile), with a multivariate risk estimate of 2.6 (95% CI, 1.5-4.4); and an erythrocyte sedimentation rate greater than 30 mm/h (75th quartile), with a multivariate risk estimate of 1.7 (95% CI, 1.1-2.8).
  
  
  CONCLUSIONS
  There is a considerable case-fatality rate in patients with BP. Older patients who require a higher dosage of oral glucocorticosteroids at hospital discharge and who have low serum albumin levels are at greater risk of death within the first year after hospitalization. These prognostic factors should be considered in the care of patients with BP as well as in the design of future clinical trials.
authors.affiliation: Department of Dermatology, Fakultät für Klinische Medizin Mannheim der Universität Heidelberg, Mannheim, Germany. berthold.rzany@charite.de
authors.forename: Berthold
authors.initials: B
authors.lastname: Rzany
authors.forename: Karin
authors.initials: K
authors.lastname: Partscht
authors.forename: Martin
authors.initials: M
authors.lastname: Jung
authors.forename: Werner
authors.initials: W
authors.lastname: Kippes
authors.forename: Dieter
authors.initials: D
authors.lastname: Mecking
authors.forename: Bozena
authors.initials: B
authors.lastname: Baima
authors.forename: Christin
authors.initials: C
authors.lastname: Prudlo
authors.forename: Beata
authors.initials: B
authors.lastname: Pawelczyk
authors.forename: Elisabeth M
authors.initials: EM
authors.lastname: Messmer
authors.forename: Monika
authors.initials: M
authors.lastname: Schuhmann
authors.forename: Ronald
authors.initials: R
authors.lastname: Sinkgraven
authors.forename: Lutz
authors.initials: L
authors.lastname: Büchner
authors.forename: Lioba
authors.initials: L
authors.lastname: Büdinger
authors.forename: Christine
authors.initials: C
authors.lastname: Pfeiffer
authors.forename: Michael
authors.initials: M
authors.lastname: Sticherling
authors.forename: Michael
authors.initials: M
authors.lastname: Hertl
authors.forename: Hans-Wilhelm
authors.initials: HW
authors.lastname: Kaiser
authors.forename: Michael
authors.initials: M
authors.lastname: Meurer
authors.forename: Detlef
authors.initials: D
authors.lastname: Zillikens
authors.forename: Gerald
authors.initials: G
authors.lastname: Messer
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D002423:Cause of Death; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000008:administration & dosage / Q000009:adverse effects*; D006801:Humans; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D010391:Pemphigoid, Bullous / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality*; D011239:Prednisolone / Q000008:administration & dosage / Q000009:adverse effects; D012189:Retrospective Studies; D012307:Risk Factors; D012709:Serum Albumin / Q000378:metabolism*; D016019:Survival Analysis
chemical_list: D005938:Glucocorticoids; D012709:Serum Albumin; D011239:Prednisolone