Assessment of healthcare costs for patients with pemphigus and bullous pemphigoid in an academic centre in Germany

Ständer, Sascha; Färber, Benedikt; Radeke, S; Schmidt, Enno; Zillikens, Detlef; Ludwig, Ralf J.

doi:10.1111/bjd.18731

Cited by 15 publications

(16 citation statements)

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“…Bullous pemphigoid (BP) is a rare autoimmune disease, characterized by blistering of the skin. It is predominantly a disease of the elderly, and is associated with high morbidity, mortality and significant healthcare costs 1,2. A previous study of BP in the UK estimated that the incidence was increasing by 17% per annum between 1996 and 2006 3.…”

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confidence: 99%

Incidence, prevalence and mortality of bullous pemphigoid in England 1998–2017: a population‐based cohort study*

et al. 2020

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Summary Background A rising incidence and high mortality were found for bullous pemphigoid (BP) over a decade ago in the UK. Updated estimates of its epidemiology are required to understand the healthcare needs of an ageing population. Objectives To determine the incidence, prevalence and mortality rates of BP in England from 1998 to 2017. Methods We conducted a cohort study of longitudinal electronic health records using the Clinical Practice Research Datalink and linked Hospital Episode Statistics. Incidence was calculated per 100 000 person‐years and annual point prevalence per 100 000 people. Multivariate analysis was used to determine incidence rate ratios by sociodemographic factors. Mortality was examined in an age‐, sex‐ and practice‐matched cohort, using linked Office of National Statistics death records. Hazard ratios (HRs) were stratified by matched set. Results The incidence was 7·63 [95% confidence interval (CI) 7·35–7·93] per 100 000 person‐years and rose with increasing age, particularly for elderly men. The annual increase in incidence was 0·9% (95% CI 0·2–1·7). The prevalence almost doubled over the observation period, reaching 47·99 (95% CI 43·09–53·46) per 100 000 people and 141·24 (95% CI 125·55–158·87) per 100 000 people over the age of 60 years. The risk of all‐cause mortality was highest in the 2 years after diagnosis (HR 2·96; 95% CI 2·68–3·26) and remained raised thereafter (HR 1·54; 95% CI 1·36–1·74). Conclusions We report a modest increase in the incidence rate of BP, but show that the burden of disease in the elderly population is considerable. Mortality is high, particularly in the first 2 years after diagnosis.

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confidence: 99%

Incidence, prevalence and mortality of bullous pemphigoid in England 1998–2017: a population‐based cohort study*

et al. 2020

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“…In individual patients, the specific pemphigus or pemphigoid disease is diagnosed based on the clinical presentation, the detection of tissue-bound autoantibodies and the autoantibody specificity ( 2 – 4 ). Albeit rare, pemphigus and pemphigoid diseases impose a major disease burden with a high unmet medical need ( 5 , 6 ). The clinical hallmark of pemphigus and pemphigoid is (muco)-cutaneous blistering, which occurs intradermal in pemphigus and subepidermal in pemphigoid.…”

Section: Pemphigus and Pemphigoidmentioning

confidence: 99%

Milestones in Personalized Medicine in Pemphigus and Pemphigoid

et al. 2021

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Pemphigus and pemphigoid diseases are autoimmune bullous diseases characterized and caused by autoantibodies targeting adhesion molecules in the skin and/or mucous membranes. Personalized medicine is a new medical model that separates patients into different groups and aims to tailor medical decisions, practices, and interventions based on the individual patient`s predicted response or risk factors. An important milestone in personalized medicine in pemphigus and pemphigoid was achieved by verifying the autoimmune pathogenesis underlying these diseases, as well as by identifying and cloning several pemphigus/pemphigoid autoantigens. The latter has become the basis of the current, molecular-based diagnosis that allows the differentiation of about a dozen pemphigus and pemphigoid entities. The importance of autoantigen-identification in pemphigus/pemphigoid is further highlighted by the emergence of autoantigen-specific B cell depleting strategies. To achieve this goal, the chimeric antigen receptor (CAR) T cell technology, which is used for the treatment of certain hematological malignancies, was adopted, by generating chimeric autoantigen receptor (CAAR) T cells. In addition to these more basic science-driven milestones in personalized medicine in pemphigus and pemphigoid, careful clinical observation and epidemiology are again contributing to personalized medicine. The identification of clearly distinct clinical phenotypes in pemphigoid like the non-inflammatory and gliptin-associated bullous pemphigoid embodies a prominent instance of the latter. We here review these exciting developments in basic, translational, clinical, and epidemiological research in pemphigus and pemphigoid. Overall, we hereby aim to attract more researchers and clinicians to this highly interesting and dynamic field of research.

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“…Bullous pemphigoid (BP) is the most prevalent subepidermal autoimmune blistering disease (AIBD) worldwide ( 1 ). Recent evidence accumulated to suggest that BP confers an increasing inpatient burden ( 2 ) owing to its growing incidence ( 3 ), its increased associated mortality ( 4 , 5 ), and high healthcare costs ( 6 ). This tissue-specific autoimmune disease arises from the production of IgG autoantibodies targeting the hemidesmosomal proteins BP180 and BP230 ( 7 ) and typically afflicts the elderly population ( 1 ).…”

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confidence: 99%

The Bidirectional Association Between Bullous Pemphigoid and Psoriasis: A Population-Based Cohort Study

et al. 2020

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The risk of developing psoriasis during the course of bullous pemphigoid (BP) is yet to be investigated. We aimed to assess the risk of psoriasis among patients with BP and the risk of BP in individuals with a history of psoriasis. A population-based retrospective cohort study was conducted comparing BP patients ( n = 3,924) with age-, sex-, and ethnicity-matched control subjects ( n = 19,280) with regard to incident cases of psoriasis. A case-control design was additionally followed to estimate the risk of BP in those with a preceding diagnosis of psoriasis. Adjusted hazard ratios (HRs) and adjusted odds ratios (ORs) were estimated by Cox regression and logistic regression, respectively. The incidence of psoriasis was 1.78 (95% CI, 1.25–2.48) and 0.67 (95%CI, 0.53–0.83) per 1,000 person-years among patients with BP and controls, respectively. Patients with BP were 2.6-fold more likely to develop psoriasis (HR, 2.60; 95%CI, 1.59–4.27) compared to controls. Furthermore, the prevalence of preexisting psoriasis was higher in patients with BP than in control subjects (1.7 vs. 1.1%, respectively; P < 0.001). A history of psoriasis was associated with a 50% increase in the risk of BP (OR, 1.53; 95%CI, 1.17–2.02). Patients with a dual diagnosis of BP and psoriasis were younger, had higher prevalence of smoking and hypertension, and were treated more frequently with prolonged systemic and topical corticosteroids when compared to the remaining patients with BP. To conclude, a bidirectional association exists between BP and psoriasis. Awareness of this association may be of great importance for physicians managing patients with BP and psoriasis.

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Assessment of healthcare costs for patients with pemphigus and bullous pemphigoid in an academic centre in Germany

Cited by 15 publications

References 5 publications

Incidence, prevalence and mortality of bullous pemphigoid in England 1998–2017: a population‐based cohort study*

Incidence, prevalence and mortality of bullous pemphigoid in England 1998–2017: a population‐based cohort study*

Milestones in Personalized Medicine in Pemphigus and Pemphigoid

The Bidirectional Association Between Bullous Pemphigoid and Psoriasis: A Population-Based Cohort Study

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