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Increased Mortality in Patients with Bullous Pemphigoid : A Nationwide Population-based Cohort Study of 5,738 Patients in Sweden

Albadri, Zeyad ; Häbel, Henrike ; Thorslund, Kristofer ; Grönhagen, Carina LU and Seifert, Oliver (2026) In Acta Dermato-Venereologica 106.
Abstract
Bullous pemphigoid (BP) is the
most common autoimmune blistering disease and has been linked to
increased mortality, although previous studies have generally relied on
small populations and no data have been available specifically for the
Swedish population. This study aimed to evaluate the 1-and 10-year
mortality rates and all-cause mortality in Swedish patients with BP
compared with the general population. Using the National Patient
Register, we conducted a retrospective cohort study and identified all
BP cases in Sweden from 1 January 2005, to 31 December 2016. Age, sex,
and county of residence matched controls without BP were drawn from the
general population, and mortality data were... (More)
Bullous pemphigoid (BP) is the
most common autoimmune blistering disease and has been linked to
increased mortality, although previous studies have generally relied on
small populations and no data have been available specifically for the
Swedish population. This study aimed to evaluate the 1-and 10-year
mortality rates and all-cause mortality in Swedish patients with BP
compared with the general population. Using the National Patient
Register, we conducted a retrospective cohort study and identified all
BP cases in Sweden from 1 January 2005, to 31 December 2016. Age, sex,
and county of residence matched controls without BP were drawn from the
general population, and mortality data were obtained from the Swedish
Cause of Death Register. In total, 5,738 patients with BP and 17,167
controls were included. BP patients had a significantly higher all-cause
mortality (HR 2.15, 95% CI 2.06–2.24) than controls, with 1-and 10-year
mortality rates of 21.2% and 80.2%, respectively. Cardiovascular
diseases accounted for the largest proportion of deaths (25.3%).
Notably, improved survival was observed among BP patients treated with
methotrexate (MTX), prednisolone, and potent topical corticosteroids (HR
0.76, 95% CI 0.69–0.83). To our knowledge, this is the first nationwide
Swedish study on BP mortality and one of the largest to date,
confirming a significantly increased mortality risk in BP patients,
particularly due to cardiovascular causes, while also suggesting that
combined MTX, prednisolone, and potent topical steroid treatment may
improve survival outcomes. (Less)
Please use this url to cite or link to this publication:
author
; ; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
autoimmune blistering disease, autoimmune subepidermal bullous disease, bullous pemphigoid, epidemiology, mortality, public health
in
Acta Dermato-Venereologica
volume
106
article number
adv44309
publisher
Medical Journals Limited
external identifiers
  • pmid:42206441
  • scopus:105040241607
ISSN
0001-5555
DOI
10.2340/actadv.v106.44309
language
English
LU publication?
yes
id
eeba53a8-88af-44b3-8bea-b37237b38ba2
date added to LUP
2026-07-02 14:53:35
date last changed
2026-09-10 20:51:51
@article{eeba53a8-88af-44b3-8bea-b37237b38ba2,
  abstract     = {{Bullous pemphigoid (BP) is the <br>
most common autoimmune blistering disease and has been linked to <br>
increased mortality, although previous studies have generally relied on <br>
small populations and no data have been available specifically for the <br>
Swedish population. This study aimed to evaluate the 1-and 10-year <br>
mortality rates and all-cause mortality in Swedish patients with BP <br>
compared with the general population. Using the National Patient <br>
Register, we conducted a retrospective cohort study and identified all <br>
BP cases in Sweden from 1 January 2005, to 31 December 2016. Age, sex, <br>
and county of residence matched controls without BP were drawn from the <br>
general population, and mortality data were obtained from the Swedish <br>
Cause of Death Register. In total, 5,738 patients with BP and 17,167 <br>
controls were included. BP patients had a significantly higher all-cause<br>
 mortality (HR 2.15, 95% CI 2.06–2.24) than controls, with 1-and 10-year<br>
 mortality rates of 21.2% and 80.2%, respectively. Cardiovascular <br>
diseases accounted for the largest proportion of deaths (25.3%). <br>
Notably, improved survival was observed among BP patients treated with <br>
methotrexate (MTX), prednisolone, and potent topical corticosteroids (HR<br>
 0.76, 95% CI 0.69–0.83). To our knowledge, this is the first nationwide<br>
 Swedish study on BP mortality and one of the largest to date, <br>
confirming a significantly increased mortality risk in BP patients, <br>
particularly due to cardiovascular causes, while also suggesting that <br>
combined MTX, prednisolone, and potent topical steroid treatment may <br>
improve survival outcomes.}},
  author       = {{Albadri, Zeyad and Häbel, Henrike and Thorslund, Kristofer and Grönhagen, Carina and Seifert, Oliver}},
  issn         = {{0001-5555}},
  keywords     = {{autoimmune blistering disease; autoimmune subepidermal bullous disease; bullous pemphigoid; epidemiology; mortality; public health}},
  language     = {{eng}},
  publisher    = {{Medical Journals Limited}},
  series       = {{Acta Dermato-Venereologica}},
  title        = {{Increased Mortality in Patients with Bullous Pemphigoid : A Nationwide Population-based Cohort Study of 5,738 Patients in Sweden}},
  url          = {{http://dx.doi.org/10.2340/actadv.v106.44309}},
  doi          = {{10.2340/actadv.v106.44309}},
  volume       = {{106}},
  year         = {{2026}},
}