Bullous Pemphigoid-Associated Mortality Rate in a Canadian Tertiary Referral Centre.

BACKGROUND: Bullous pemphigoid (BP) is the most common autoimmune blistering disorder in adults. Most individuals with BP are over the age of 60. Its worldwide incidence has been increasing owing to population aging. Observational studies published over the last 2 decades highlight the non-negligible, albeit variable overall mortality of BP patients, with reported 12-month mortality rates of 10.8% to 40.8%, and 24-month mortality rates of 20.1% to 51.0%. Data in the Canadian population are lacking. OBJECTIVES: We aimed to estimate the 12- and 24-month overall mortality rate of Canadian patients diagnosed with BP, and to identify independent risk factors adversely impacting overall survival. METHODS: A retrospective cohort study of 166 patients with a diagnosis of BP between 2010 and 2020 was carried out at Centre hospitalier de l'Université de Montréal (CHUM), a tertiary referral center in Montréal, Québec, Canada. Cumulative mortality was calculated using the Kaplan-Meier estimator, and independent prognostic factors were identified using a Cox proportional hazards regression model. RESULTS: Eighty-five patients (51.2%) in our study were female. The median age was 79.1 years old, and 80 patients (48.2%) were 80 years old or older. Mortality at 12 and 24 months in our study cohort was 16.2% (CI95% = 10.5 - 21.8) and 27.6% (CI95% = 20.5 - 34.7), respectively. In a multivariate analysis, patients who were male, 80 years old or older, and/or had a diagnosis of a major neurocognitive disorder had a poorer overall survival. CONCLUSIONS: The all-cause mortality of patients with BP in our study population compared favorably with international data reported in the literature.

Increased frequency of multiple sclerosis among patients with bullous pemphigoid: a population-based cohort study on comorbidities anchored around the diagnosis of bullous pemphigoid.

BACKGROUND: Bullous pemphigoid (BP) is a disease of the elderly and may be associated with neurological and cardiovascular diseases and diabetes. Mortality rates strongly exceed those of the background population. OBJECTIVES: To investigate the frequency of comorbidities and their temporal relation to BP. METHODS: A register-based matched-cohort study on all Danish patients with a hospital-based diagnosis of BP (n = 3281). The main outcomes were multiple sclerosis (MS), Parkinson disease (PD), Alzheimer disease (AD), stroke, diabetes types 1 and 2, malignancies, ischaemic heart disease (IHD), hypertension and eventually death. RESULTS: At baseline, patients with BP had increased prevalences of MS [odds ratio (OR) 9·7, 95% confidence interval (CI) 6·0-15·6], PD (OR 4·2, 95% CI 3·1-5·8), AD (OR 2·6, 95% CI 1·8-3·5) and stroke (OR 2·7, 95% CI 2·4-2·9). Furthermore, malignancies, cardiovascular disease and diabetes were over-represented among patients with BP: type 1 diabetes (OR 3·1, 95% CI 2·5-3·8), type 2 diabetes (OR 2·3, 95% CI 2·0-2·6), malignancies (OR 1·3, 95% CI 1·1-1·4), IHD (OR 1·7, 95% CI 1·5-1·9) and hypertension (OR 2·0, 95% CI 1·8-2·2). During follow-up, the risk of MS was significantly higher among patients with BP [hazard ratio (HR) 9·4, 95% CI 4·9-18·0], even if events during the first year after diagnosis of BP were excluded (HR 5·1, 95% CI 2·3-11·3). Patients with BP had an average increased mortality rate of 2·04 (95% CI 1·96-2·13). CONCLUSIONS: We discovered a significantly increased frequency of MS among patients with BP. At the time of diagnosis, patients with BP had an excessive number of comorbidities and an increased mortality rate over the following years.

Risk of Death in Bullous Pemphigoid: A Retrospective Database Study in Finland.

Bullous pemphigoid (BP) is an autoimmune skin disease of elderly people, which is associated with increased mortality. The aim of this study was to investigate the standardized mortality ratio (SMR) for BP in Finland, and concomitant comorbidities and medications. This was a retrospective database study of all cases of BP diagnosed at the Department of Dermatology, Oulu University Hospital, Finland, between 1985 and 2012. A total of 198 immunologically confirmed cases of BP were found. One-year mortality was 16.7%, and SMR 7.56 (95% confidence interval (CI) 4.98-10.14). The most common comorbidities were cardiovascular diseases (76.3%) and neurodegenerative diseases (40.9%). Malignancies (8.6%) were associated with increased mortality (hazard ratio = 2.4, 95% CI 1.1-5.5, p = 0.047). A novel finding was that polypharmacy was very common in patients with BP, and the higher the number of drugs, the greater the mortality. In conclusion, the mortality for BP in Finland is 7.6-fold that of a reference population, and malignancies and polypharmacy are associated with increased mortality.

[Changes in mortality in the Reims University Hospital Dermatology Department (1996-2009)]. / Évolution de la mortalité dans le service de dermatologie du CHU de Reims (1996-2009).

BACKGROUND: End-of-life care is becoming a daily concern of French dermatology departments. The aim of this single-centre retrospective study was to assess changes in mortality and end-of-life care in a university dermatology department over a 14-year period. PATIENTS AND METHODS: The medical records of all patients dying in our dermatology department from 1996 to 2009 were studied retrospectively. A descriptive analysis was performed together with a comparison between the two periods demarcated by the institution of the French National Cancer Plan at the end of 2002. RESULTS: The number of patients dying in the department rose by 33% from 108 for period 1 (1996-2002) to 144 for period 2 (2003-2009). The majority of patients presented metastatic melanoma, with doubling of numbers between the two periods. During period 2, 40 % of patients were managed in collaboration with the mobile palliative care team. CONCLUSION: This study shows an increase in the number of deaths in a French university hospital dermatology department over the 14-year period in question, ascribable mainly to an increase in the number of patients receiving end-of-life care for metastatic melanoma. Significant changes were noted in the management of these patients with an increase in palliative care procedures.

Mortality rate in bullous pemphigoid: a retrospective monocentric cohort study.

BACKGROUND: Bullous pemphigoid (BP) is the most common autoimmune blistering skin disease and is associated with an increased mortality. The end points of our study were to evaluate the mortality rate in a retrospective cohort of BP patients followed up to 5 years after the diagnosis and to determine prognostics factors. METHODS: All new cases of BP diagnosed between 1990 and 2003 in the University Hospital of Geneva were retrospectively collected. 60 patients were included, 47 (88.6%) of whom were treated with a combination of corticosteroids and chlorambucil. RESULTS: The 1-year, 2-year and 5-year probabilities of death were 26.7, 37.1 and 60.8%, respectively. Old age, dementia and use of chlorambucil at initial doses of 6 mg/day, but not at lower doses, were associated with poor prognosis in multivariate analysis. CONCLUSION: Our study confirms that BP is associated with a high mortality. The observed mortality rates are however higher than those of previous studies, which is probably related to the inclusion of more debilitated patients.