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Risk Factors for Lethal Outcome in Patients With Bullous Pemphigoid
Low Serum Albumin Level, High Dosage of Glucocorticosteroids, and Old Age
Berthold Rzany, MD, ScM;
Karin Partscht, MD;
Martin Jung, MD;
Werner Kippes, MD;
Dieter Mecking, MD;
Bozena Baima, MD;
Christin Prudlo, MD;
Beata Pawelczyk, MD;
Elisabeth M. Messmer, MD;
Monika Schuhmann, MD;
Ronald Sinkgraven, BS;
Lutz Büchner, MD;
Lioba Büdinger, MD;
Christine Pfeiffer, MD;
Michael Sticherling, MD;
Michael Hertl, MD;
Hans-Wilhelm Kaiser, MD;
Michael Meurer, MD;
Detlef Zillikens, MD;
Gerald Messer, MD
Arch Dermatol. 2002;138:903-908.
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.
Design A multicenter retrospective cohort study.
Setting Seven dermatologic university hospitals in Germany.
Participants A total of 369 patients diagnosed as having BP between January 1, 1987,
and December 31, 1997.
Statistics 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.
From the Department of Dermatology, Fakultät für Klinische
Medizin Mannheim der Universität Heidelberg, Mannheim (Drs Rzany, Jung,
and Schuhmann and Mr Sinkgraven); Departments of Dermatology (Drs Partscht
and Messer) and Ophthalmology (Dr Messmer), Ludwig-Maximilians-Universität,
München; Department of Dermatology, Universität Würzburg, Würzburg
(Drs Kippes and Zillikens); Department of Dermatology, Universität Bonn,
Bonn (Drs Mecking and Kaiser); Department of Dermatology, Universität
Kiel, Kiel (Drs Baima and Sticherling); Department of Dermatology, Technische
Universität Dresden, Dresden (Drs Prudlo, Büchner, Pfeiffer, and
Meurer); and Department of Dermatology, Klinikum der Rheinisch-Westfälische
Technischen Hochschule Aachen, Aachen (Drs Pawelczyk, Büdinger, and Hertl),
Germany. Dr Rzany is now with the Center for Evidence Based Medicine in Dermatology,
Department of Dermatology, Charité University Hospital, Berlin; Dr
Sticherling, with the Department of Dermatology, Universität Leipzig,
Leipzig; and Dr Hertl, with the Department of Dermatology, Universität
Erlangen-Nürnberg, Erlangen, Germany.
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