{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,28]],"date-time":"2026-05-28T10:04:14Z","timestamp":1779962654942,"version":"3.53.1"},"reference-count":41,"publisher":"MJS Publishing, Medical Journals Sweden AB","license":[{"start":{"date-parts":[[2026,5,28]],"date-time":"2026-05-28T00:00:00Z","timestamp":1779926400000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Acta Derm Venereol"],"abstract":"<jats:p>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\u20132.24) 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\u20130.83). 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.<\/jats:p>","DOI":"10.2340\/actadv.v106.44309","type":"journal-article","created":{"date-parts":[[2026,5,28]],"date-time":"2026-05-28T09:27:20Z","timestamp":1779960440000},"page":"adv44309","source":"Crossref","is-referenced-by-count":0,"title":["Increased Mortality in Patients with Bullous Pemphigoid: A Nationwide Population-based Cohort Study of 5,738 Patients in Sweden"],"prefix":"10.2340","volume":"106","author":[{"given":"Zeyad","family":"Albadri","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henrike","family":"H\u00e4bel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kristofer","family":"Thorslund","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carina","family":"Gr\u00f6nhagen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Oliver","family":"Seifert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1183","published-online":{"date-parts":[[2026,5,28]]},"reference":[{"key":"157249","doi-asserted-by":"crossref","unstructured":"Zillikens D, Wever S, Roth A, Weidenthaler-Barth B, Hashimoto T, Br\u00f6cker EB. 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Arch Dermatol Res 2017; 309: 721\u2013727.","DOI":"10.1007\/s00403-017-1778-4"},{"key":"157259","doi-asserted-by":"crossref","unstructured":"Colbert RL, Allen DM, Eastwood D, Fairley JA. Mortality rate of bullous pemphigoid in a US medical center. J Invest Dermatol 2004; 122: 1091\u20131095.","DOI":"10.1111\/j.0022-202X.2004.22504.x"},{"key":"157260","doi-asserted-by":"crossref","unstructured":"Roujeau JC, Lok C, Bastuji-Garin S, Mhalla S, Enginger V, Bernard P. High risk of death in elderly patients with extensive bullous pemphigoid. Arch Dermatol 1998; 134: 465\u2013469.","DOI":"10.1001\/archderm.134.4.465"},{"key":"157261","doi-asserted-by":"crossref","unstructured":"Fine JD. Management of acquired bullous skin diseases. N Engl J Med 1995; 333: 1475\u20131484.","DOI":"10.1056\/NEJM199511303332207"},{"key":"157262","unstructured":"Korman NJ. Bullous pemphigoid: the latest in diagnosis, prognosis, and therapy. Arch Dermatol 1998; 134: 1137\u20131141."},{"key":"157263","doi-asserted-by":"crossref","unstructured":"Borradori L, Van Beek N, Feliciani C, et al. Updated S2 K guidelines for the management of bullous pemphigoid initiated by the European Academy of Dermatology and Venereology (EADV). J Eur Acad Dermatol Venereol 2022; 36: 1689\u20131704.","DOI":"10.1111\/jdv.18220"},{"key":"157264","doi-asserted-by":"crossref","unstructured":"Ludvigsson JF, Otterblad-Olausson P, Pettersson BU, Ekbom A. The Swedish personal identity number: possibilities and pitfalls in healthcare and medical research. Eur J Epidemiol 2009; 24: 659\u2013667.","DOI":"10.1007\/s10654-009-9350-y"},{"key":"157265","doi-asserted-by":"crossref","unstructured":"Ludvigsson JF, Andersson E, Ekbom A, et al. External review and validation of the Swedish national inpatient register. 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Prediction of survival for patients with bullous pemphigoid: a prospective study. Arch Dermatol 2005; 141: 691\u2013698.","DOI":"10.1001\/archderm.141.6.691"},{"key":"157273","doi-asserted-by":"crossref","unstructured":"Rzany B, Partscht K, Jung M, et al. Risk factors for lethal outcome in patients with bullous pemphigoid: low serum albumin level, high dosage of glucocorticosteroids, and old age. Arch Dermatol 2002; 138: 903\u2013908.","DOI":"10.1001\/archderm.138.7.903"},{"key":"157274","doi-asserted-by":"crossref","unstructured":"Doffoel-Hantz V, Sparsa A, Marin B, Durox H, Bonnetblanc JM, B\u00e9dane C. Profil \u00e9volutif des patients atteints de pemphigo\u00efde bulleuse au cours de la premi\u00e8re ann\u00e9e de traitement. Ann Dermatol Venereol 2009; 136: 407\u2013411.","DOI":"10.1016\/j.annder.2009.01.009"},{"key":"157275","unstructured":"F\u00f6rsti AK, Jokelainen J, Timonen M, Tasanen K. Risk of death in bullous pemphigoid: a retrospective database study in Finland. Acta Derm Venereol 2016; 96: 758\u2013761."},{"key":"157276","doi-asserted-by":"crossref","unstructured":"Kridin K, Shihade W, Bergman R. Mortality in patients with bullous pemphigoid: a retrospective cohort study, systematic review and meta-analysis. Acta Derm Venereol 2019; 99: 72\u201377.","DOI":"10.2340\/00015555-2930"},{"key":"157277","doi-asserted-by":"crossref","unstructured":"Bardazzi F, Filippi F, Chessa MA, et al. Mortality and prognostic factors in patients with bullous pemphigoid: a retrospective multicenter Italian study. J Eur Acad Dermatol Venereol 2022; 36: 2473\u20132481.","DOI":"10.1111\/jdv.18441"},{"key":"157278","doi-asserted-by":"crossref","unstructured":"Shen WC, Chiang HY, Chen PS, Lin YT, Kuo CC, Wu PY. Risk of all-cause mortality, cardiovascular disease mortality, and cancer mortality in patients with bullous pemphigoid. 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Br J Dermatol 2009; 160: 1343\u20131345.","DOI":"10.1111\/j.1365-2133.2009.09122.x"},{"key":"157285","doi-asserted-by":"crossref","unstructured":"Kridin K, Bieber K, Vorobyev A, Moderegger EL, Hernandez G, Schmidt E, et al. Risk of death, major adverse cardiac events and relapse in patients with bullous pemphigoid treated with systemic or topical corticosteroids. Br J Dermatol 2024; 191: 539\u2013547.","DOI":"10.1093\/bjd\/ljae219"},{"key":"157286","doi-asserted-by":"crossref","unstructured":"Karakioulaki M, Eyerich K, Patsatsi A. Advancements in bullous pemphigoid treatment: a comprehensive pipeline update. Am J Clin Dermatol 2024; 25: 195\u2013212.","DOI":"10.1007\/s40257-023-00832-1"},{"key":"157287","doi-asserted-by":"crossref","unstructured":"Williams HC, Wojnarowska F, Kirtschig G, et al. Doxycycline versus prednisolone as an initial treatment strategy for bullous pemphigoid: a pragmatic, non-inferiority, randomised controlled trial. 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