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Mortality in bullous pemphigoid and prognostic factors in 1st and 3rd year of follow-up in specialized centre in Poland

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Abstract

Bullous pemphigoid (BP) is associated with higher mortality and coexisting comorbidities, some of them affecting poor prognosis. The aim of the study was to identify prognostic factors causing greater mortality both in the 1st and 3rd year of follow-up and to determine the 1-, 2-, 3-year mortality rates, standardized mortality ratio (SMR) in Polish BP patients. All patients with BP (a cohort of 205 patients, mean age 76.2 years) diagnosed between 5 January 2000 and 10 December 2013 in a referral unit for autoimmune bullous diseases at the university hospital in Poland were included retrospectively. Mortality data were obtained from the Centre for Document Personalization at the Minister of Interior and Administration. Our original observation was that prednisone in moderate dose (0.5 mg kg−1) in monotherapy was an independent risk factor of fatal prognosis in the 1st year of follow-up, assessed using multivariate analysis. We confirmed the strong correlation between neurological diseases and greater mortality. Both in the 1st and 3rd year of follow-up, dementia and Parkinson disease resulted in increased mortality. We also found that arrhythmias significantly increased mortality in the 1st and 3rd year of follow-up. The prognostic factors in BP changed over time of follow-up. In the 3rd year of observation, the age above 77, longer hospitalization and BP severity were associated with greater mortality. We observed poorer prognosis in BP patients than age-matched general Polish population. The 1-, 2-, 3-year mortality rates were 22.4, 31.2, 39.5% and SMR was 3.8 (95% CI 3.4–3.7).

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Acknowledgements

This study was supported by a Grant from the National Centre of Science, Poland, Project no. 2P05B 065 30.

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Correspondence to Agnieszka Kalinska-Bienias.

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The authors declare that they have no conflict of interest.

Funding

This study was supported by a grant from National Centre of Science, Poland, Project no. 2P05B 065 30.

Ethical approval

All procedures performed in the study involving human participants were in accordance with the ethical standards of the Bioethical Committee of Medical University of Warsaw and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Data were obtained from the Centre for Document Personalization at the Minister of Interior and Administration.

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Kalinska-Bienias, A., Lukowska-Smorawska, K., Jagielski, P. et al. Mortality in bullous pemphigoid and prognostic factors in 1st and 3rd year of follow-up in specialized centre in Poland. Arch Dermatol Res 309, 709–719 (2017). https://doi.org/10.1007/s00403-017-1772-x

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  • DOI: https://doi.org/10.1007/s00403-017-1772-x

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