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

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) 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).

Comorbidities and Treatment Strategies in Bullous Pemphigoid: An Appraisal of the Existing Litterature

“…Similarly, Kalinska-Bienias et al found 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 by multivariate analysis ( 25 ). Patients treated with prednisone in monotherapy were in this study associated with almost a two-time increased risk of mortality.…”

Clinical characteristics, mortality, and prognostic factors for bullous pemphigoid in a Thai population

“…Previous studies have reported that certain comorbidities, including neurological diseases, particularly dementia, Parkinson's disease, and stroke, and modalities of treatment, such as the use of corticosteroids or immunosuppressive drugs, and high serum anti-BP180 IgG levels may affect the mortality rate. [ 6 , 21 – 24 ] Due discrepancies in the mortality rates reported among various countries and the lack of studies regarding the mortality rate among the Thai population, we aimed to determine the mortality rate among patients with BP in a representative Thai cohort, from the largest tertiary academic center in Southern Thailand, to identify the factors affecting survival.…”

The association between neurological diseases, malignancies and cardiovascular comorbidities among patients with bullous pemphigoid: Case-control study in a specialized Polish center

“…Bullous pemphigoid is a potentially fatal disease with 1-year and 3-year mortality rates assessed at 22.4% and 39.5%, respectively, in Polish BP patients. 3,4 In recent years, many epidemiological studies have investigated the coexistence of BP with various comorbidities. In fact, the most intense research has been focused on neurological diseases (NDs), showing that BP patients are more likely to have any type of ND.…”