Key points Do patients with bullous pemphigoid (BP) have an increased risk of mortality from cardiovascular disease (CVD)? Findings In this cohort study, 252 patients with AP confirmed by clinical, pathological, or immunofluorescent evidence were individually matched with 1008 patients without AP according to age, sex, and date of dermatology clinic visit. Bullous pemphigoid was associated with a 5-fold increased risk of CVD mortality at 1, 3, and 5 years after adjustment for comorbidities; use of antidiabetic agents, insulin and corticosteroids; glucose level; and white blood cell count. Meaning These findings suggest that more evidence is needed to determine guidance to optimize CVD protection for patients with BP. |
Importance
The role of bullous pemphigoid (BP) in cardiovascular disease (CVD) mortality remains controversial, and analyzes of causes of death among BP patients based on individual data are still lacking.
Aim
To assess the risk of all-cause mortality, CVD mortality, and cancer mortality in patients with AP.
Design, scope and participants
This cohort study identified patients who received a diagnosis and treatment for BP during their visits to the dermatology clinic at a tertiary medical center in central Taiwan between January 1, 2007 and December 31, 2017.
The controls were patients without AP and were individually matched to the cases (4:1) according to age, sex, and date of visit to the dermatology clinic. Data was analyzed from March 6, 2019 to April 2, 2021.
Exhibitions
Bullous pemphigoid was confirmed pathologically with typical direct immunofluorescence findings or clinically with typical clinical presentation, positive findings of a basement membrane zone antibody test, and corticosteroid use for at least 28 cumulative days.
Main results and measures
Mortality results confirmed by the National Death Registry.
Results
Of 252 BP patients and 1008 matched control patients (N = 1260), 685 (54.4%) were men and the median age was 78.0 (IQR, 70.3-84.8) years. Patients with BP had higher CVD mortality at 1 year (20 [7.9%] vs 13 [1.3%]), at 3 years (28 [11.1%] vs 24 [2.4%] %]) and at 5 years (31 [12.3%] vs 39 [3.9%]) compared with matched control patients.
After adjusting for potential confounding variables, patients with BP had a 5-fold increased risk of CVD mortality at 1 year (hazard ratio [HR], 5.29 [95% CI, 2.40-11.68]), at 3 years (HR, 5.79 [95% CI, 3.11-10.78]) and 5 years (HR, 4.95 [95% CI, 2.88-8.51]).
Subgroup analyzes revealed that the risk of CVD mortality associated with BP was higher in patients without a history of hypertension (HR, 7.28 [95% CI, 3.87-13.69]) or CVD (HR, 6.59 [95% CI, 3.40-12.79]) and in patients without prior diuretic use (HR, 5.75 [95% CI, 3.15-10.50]) compared with patients paired control units.
Furthermore, all-cause mortality associated with BP was higher in patients without prior corticosteroid use than in control patients (HR, 5.65 [95% CI, 4.19-7.61]).
Conclusions and relevance The findings of this cohort study suggest that BP was associated with a 5-fold increased risk of CVD mortality, particularly in patients without underlying hypertension or CVD or those without prior use of corticosteroids or diuretics. Future studies should investigate the benefits of routine monitoring and timely management of CVD symptoms and signs in patients with BP. |
