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Bullous pemphigoid and pemphigus vulgaris--incidence and mortality in the UK: population based cohort study.

Langan SM, Smeeth L, Hubbard R, Fleming KM, Smith CJ, West J - BMJ (2008)

Bottom Line: For pemphigus vulgaris, the risk of death was three times greater than for controls (adjusted hazard ratio=3.3, 2.2 to 5.2).Both disorders are associated with a high risk of death.Previous estimates may have underestimated the risk of death associated with these diseases.

View Article: PubMed Central - PubMed

Affiliation: Centre of Evidence-based Dermatology, University of Nottingham, Queen's Medical Centre, Nottingham NG7 2UH. sinead.langan@nottingham.ac.uk

ABSTRACT

Objective: To determine the incidence of and mortality from bullous pemphigoid and pemphigus vulgaris in the United Kingdom.

Design: Retrospective historical cohort study.

Setting: Computerised medical records from the health improvement network, a large population based UK general practice database.

Participants: Patients with pemphigus vulgaris and bullous pemphigoid diagnostic codes and age, sex, and practice matched controls.

Main outcome measures: Incidence and mortality compared with the control population by calendar period, age group, sex, geographical region, and degree of social deprivation.

Results: 869 people with bullous pemphigoid and 138 people with pemphigus vulgaris were identified. The median age at presentation for bullous pemphigoid was 80 (range 23-102) years, and 534 (61%) patients were female. The median age at presentation for pemphigus vulgaris was 71 (21-102) years, and 91 (66%) patients were female. Incidences of bullous pemphigoid and pemphigus vulgaris were 4.3 (95% confidence interval 4.0 to 4.6) and 0.7 (0.6 to 0.8) per 100 000 person years. The incidence of bullous pemphigoid increased over time; the average yearly increase was 17% (incidence rate ratio=1.2, 95% confidence interval 1.1 to 1.2). An average yearly increase in incidence of pemphigus vulgaris of 11% (incidence rate ratio=1.1, 1.0 to 1.2) occurred. The risk of death for patients with bullous pemphigoid was twice as great as for controls (adjusted hazard ratio=2.3, 95% confidence interval 2.0 to 2.7). For pemphigus vulgaris, the risk of death was three times greater than for controls (adjusted hazard ratio=3.3, 2.2 to 5.2).

Conclusions: Incidences of bullous pemphigoid and pemphigus vulgaris are increasing. The reasons for the changes in incidence are not clearly understood but have implications for identifying causative factors. Both disorders are associated with a high risk of death. Previous estimates may have underestimated the risk of death associated with these diseases.

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Related in: MedlinePlus

Fig 1 Age at diagnosis in patients with bullous pemphigoid and pemphigus vulgaris
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fig1: Fig 1 Age at diagnosis in patients with bullous pemphigoid and pemphigus vulgaris

Mentions: Our cohort included 869 people with bullous pemphigoid contributing 1993 person years and 3453 matched controls contributing 9765 person years. The median age at first presentation for bullous pemphigoid was 80 (range 23-102) years (fig 1), and 534 (61%) patients were women. One hundred and thirty eight people with pemphigus vulgaris contributed 380 person years, and 551 matched controls contributed 1763 person years. The median age at first presentation for pemphigus vulgaris was 71 (21-102) years (fig 1), and 91 (66%) patients were women. The median length of follow-up for people with bullous pemphigoid was 1.6 (range 0-9) years, and that for pemphigus vulgaris was 2.0 (0-10.4) years. Losses to follow-up were similar in cases and controls in both diseases (by the end of year 2, bullous pemphigoid cases and controls 34% lost; pemphigus vulgaris cases 30% lost, controls 31% lost) (figs 2 and 3).


Bullous pemphigoid and pemphigus vulgaris--incidence and mortality in the UK: population based cohort study.

Langan SM, Smeeth L, Hubbard R, Fleming KM, Smith CJ, West J - BMJ (2008)

Fig 1 Age at diagnosis in patients with bullous pemphigoid and pemphigus vulgaris
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC2483869&req=5

fig1: Fig 1 Age at diagnosis in patients with bullous pemphigoid and pemphigus vulgaris
Mentions: Our cohort included 869 people with bullous pemphigoid contributing 1993 person years and 3453 matched controls contributing 9765 person years. The median age at first presentation for bullous pemphigoid was 80 (range 23-102) years (fig 1), and 534 (61%) patients were women. One hundred and thirty eight people with pemphigus vulgaris contributed 380 person years, and 551 matched controls contributed 1763 person years. The median age at first presentation for pemphigus vulgaris was 71 (21-102) years (fig 1), and 91 (66%) patients were women. The median length of follow-up for people with bullous pemphigoid was 1.6 (range 0-9) years, and that for pemphigus vulgaris was 2.0 (0-10.4) years. Losses to follow-up were similar in cases and controls in both diseases (by the end of year 2, bullous pemphigoid cases and controls 34% lost; pemphigus vulgaris cases 30% lost, controls 31% lost) (figs 2 and 3).

Bottom Line: For pemphigus vulgaris, the risk of death was three times greater than for controls (adjusted hazard ratio=3.3, 2.2 to 5.2).Both disorders are associated with a high risk of death.Previous estimates may have underestimated the risk of death associated with these diseases.

View Article: PubMed Central - PubMed

Affiliation: Centre of Evidence-based Dermatology, University of Nottingham, Queen's Medical Centre, Nottingham NG7 2UH. sinead.langan@nottingham.ac.uk

ABSTRACT

Objective: To determine the incidence of and mortality from bullous pemphigoid and pemphigus vulgaris in the United Kingdom.

Design: Retrospective historical cohort study.

Setting: Computerised medical records from the health improvement network, a large population based UK general practice database.

Participants: Patients with pemphigus vulgaris and bullous pemphigoid diagnostic codes and age, sex, and practice matched controls.

Main outcome measures: Incidence and mortality compared with the control population by calendar period, age group, sex, geographical region, and degree of social deprivation.

Results: 869 people with bullous pemphigoid and 138 people with pemphigus vulgaris were identified. The median age at presentation for bullous pemphigoid was 80 (range 23-102) years, and 534 (61%) patients were female. The median age at presentation for pemphigus vulgaris was 71 (21-102) years, and 91 (66%) patients were female. Incidences of bullous pemphigoid and pemphigus vulgaris were 4.3 (95% confidence interval 4.0 to 4.6) and 0.7 (0.6 to 0.8) per 100 000 person years. The incidence of bullous pemphigoid increased over time; the average yearly increase was 17% (incidence rate ratio=1.2, 95% confidence interval 1.1 to 1.2). An average yearly increase in incidence of pemphigus vulgaris of 11% (incidence rate ratio=1.1, 1.0 to 1.2) occurred. The risk of death for patients with bullous pemphigoid was twice as great as for controls (adjusted hazard ratio=2.3, 95% confidence interval 2.0 to 2.7). For pemphigus vulgaris, the risk of death was three times greater than for controls (adjusted hazard ratio=3.3, 2.2 to 5.2).

Conclusions: Incidences of bullous pemphigoid and pemphigus vulgaris are increasing. The reasons for the changes in incidence are not clearly understood but have implications for identifying causative factors. Both disorders are associated with a high risk of death. Previous estimates may have underestimated the risk of death associated with these diseases.

Show MeSH
Related in: MedlinePlus