Bullous pemphigoid: Its incidence, mortality and clinical outcome in New Zealand

Abstract

Background/objectives Bullous pemphigoid (BP) is an uncommon autoimmune bullous disorder, with significant morbidity and mortality. Mortality may be as high as 23.5% in the first year after diagnosis. Clear epidemiologic data across Australasia are lacking. Methods A retrospective, multi‐centred cohort study was designed to determine the incidence and mortality of bullous pemphigoid in New Zealand. Data from all histopathologically diagnosed patients with bullous pemphigoid between 2009 and 2015 from the Auckland region were obtained. Demographics, clinical characteristics and outcome 3 years from diagnosis (until 31 December 2018) were collected. Demographic data were compared against a denominator year‐matched New Zealand Census population. Results One hundred sixty‐one patients had confirmed bullous pemphigoid, with an incidence rate of 3.03/100 000 person‐years [95% CI 2.58–3.54]; 70% were of European ethnicity; 12.4% were Pacific peoples; 11.2% were Asian; and 6.8% were Māori. 45.3% had associated cognitive impairment and/or stroke. In the 3‐year follow‐up, 25% had treatment complications mostly from prednisone therapy. The mortality rate was 40%, highest in the first year of diagnosis, with age at diagnosis a predictor. Conclusion The incidence and mortality rates are comparable to the UK/Northern Europe. Knowledge of the epidemiology of bullous pemphigoid in New Zealand and within an international settling informs the provision of future care and treatments.

The association between bullous pemphigoid and cognitive outcomes in middle-aged and older adults: A systematic review and meta-analysis

“…In this meta-analysis, we found that BP was associated with higher odds of all-cause dementia in middle-aged and older participants in both case-control and cohort studies. This finding is consistent with previous studies that reported an increased risk of dementia in patients with BP [ 1 , 2 , 8 ]. The possible mechanisms underlying this association are not fully understood, but some hypotheses have been proposed.…”

“…We aligned our inclusion and exclusion criteria with the PICO framework, which is a commonly used tool for framing systematic review research questions and developing literature search strategies. We included studies that: (1) were cohort, case-control, or cross-sectional in design; (2) reported quantitative data on the risk of cognitive outcomes in BP patients compared to a control group of non-bullous pemphigoid patients; (3) provided the effect size estimates HRs or ORs and their 95% CI; and (4) were published in English. We excluded studies that: (1) did not measure cognitive outcomes; (2) did not have a control group; (3) did not provide sufficient data for meta-analysis; (4) were case reports, reviews, meta-analyses, letters, editorials, or commentaries; or (5) had a quality score of less than 4 according to the Newcastle-Ottawa Scale (NOS).…”

“…It is characterized by the presence of autoantibodies against two hemidesmosomes proteins, BP180 and BP230, which are involved in the adhesion of epidermal cells to the basement membrane [ 1 ]. BP mainly affects elderly people, with a peak incidence between 70 and 80 years of age [ 2 ]. The clinical manifestations of BP include tense blisters, erosions, urticarial plaques and pruritus, which can have a significant impact on patients’ quality of life [ 3 ].…”

Rituximab Therapy for Persistent, Severe and Extensive Idiopathic Bullous Pemphigoid

“…Idiopathic Bullous pemphigoid (IBP) is an uncommon autoimmune bullous disorder, with significant morbidity and mortality. It has an incidence rate of 3/100,000 person-years yet a high mortality at 23.5% in the first year after diagnosis [1]. It is predominantly a disease of the elderly, and is associated with significant health care costs [2].…”