incidence and mortality of bullous pemphigoid and pemphigus vulgaris in the uk l.
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Incidence and mortality of bullous pemphigoid and pemphigus vulgaris in the UK. Sin éad Langan Joe West. Bullous pemphigoid. Incidence rates between 0.2 and 3 per 100,000 person-years Estimated incidence of 1.4 per 100,000 person-years in Scotland Reported 1-year mortality rates

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incidence and mortality of bullous pemphigoid and pemphigus vulgaris in the uk

Incidence and mortality of bullous pemphigoid and pemphigus vulgaris in the UK

Sinéad Langan

Joe West

bullous pemphigoid
Bullous pemphigoid

Incidence rates between 0.2 and 3 per

100,000 person-years

Estimated incidence of 1.4 per

100,000 person-years in Scotland

Reported 1-year mortality rates

between 6% (USA) and 41% (France)

pemphigus vulgaris
Pemphigus vulgaris

Few studies, incidence between 0.08 and

1.6 per 100,000 person-years

No data from the UK

1 year mortality rates between 4.8 and

54%; no clear estimate of overall disease-

specific mortality rates

objectives
Objectives

To determine incidence and

mortality of bullous pemphigoid and

pemphigus vulgaris in the United

Kingdom (UK)

data source
Data source

Computerised medical records from

the Health Improvement Network

(THIN), a large population-based UK

general practice database were

analysed

confounders
Confounders
  • Age group in 10 year bands
  • Gender
  • Region
  • Townsend scores
study populations
Study populations
  • All patients >20 years with bullous pemphigoid and pemphigus vulgaris diagnosed 1996-2006
  • First 3 months following registration excluded
  • 4 matched controls selected per case
incidence
Incidence
  • Incidence rates by age category, gender and calendar period
  • Incidence rate ratios (IRR) adjusting for changes in age, gender structure and calendar period over time
mortality
Mortality
  • Deaths in cases and controls
  • Kaplan-Meier techniques used to calculate 1 year mortality and 5 year survival rates
  • Cox regression to compare mortality experience of cases and controls
ons data
ONS data

Incidence and mortality rates from this

study applied to ONS population data

to calculate expected numbers of new

cases and number of deaths

bullous pemphigoid14
Bullous pemphigoid
  • 868 people with bullous pemphigoid and 3453 matched controls
  • Median (IQR) age at first presentation for bullous pemphigoid 80 (15) years
  • 534 (61%) were female
pemphigus vulgaris15
Pemphigus vulgaris
  • 139 people with pemphigus vulgaris and 551 matched controls
  • Median (IQR) age at first presentation for pemphigus vulgaris 71 (30) years
  • 91 (66%) were female
bullous pemphigoid incidence
Bullous pemphigoid incidence
  • Crude incidence= 4.3 (95% CI 4.0-4.6) per 100,000 py
  • 17% increase per year (IRR 1.2, 95% CI 1.1-1.2), adjusted for gender and age
  • No association with region or Townsend scores
bullous pemphigoid mortality
Bullous pemphigoid mortality

1-year mortality rate=19%

(95% CI 16-22)

Absolute excess mortality for

cases=70 deaths per 1000py

Hazard ratios:

Crude 2.1 (95% CI 1.8-2.4)

Adjusted 2.3 (95% CI 2.0-2.7)

pemphigus vulgaris incidence
Pemphigus vulgarisincidence
  • Crude incidence= 0.7 (95% CI 0.6-0.8) per 100,000 py
  • 11% increase per year (IRR 1.1, 95% CI 1.0-1.2), adjusted for gender and age
  • No association with region or Townsend scores
pemphigus vulgaris mortality
Pemphigus vulgarismortality

1-year mortality rate=12%

(95% CI 8-19)

Absolute excess mortality for

cases=62 deaths per 1000py

Hazard ratios:

Crude 2.8 (95% CI 1.9-4.3)

Adjusted 3.4 (95% CI 2.2-5.2)

how many new cases per year
How many new cases per year?

Bullous pemphigoid

Applied to ONS data, 2370 new cases

per year

Pemphigus vulgaris

Applied to ONS data, 467 new

Cases per year

how many deaths between 2001 and 2005
How many deaths between 2001 and 2005?

Bullous pemphigoid

Estimated 1977 deaths over 5

years (vs. 190, ONS)

Pemphigus vulgaris

Estimated 221 deaths over

5 years (vs. 36, ONS)

key findings
Key findings
  • Increased incidence both diseases between 1996 and 2006
  • No reduction in mortality rates
  • Therefore unlikely to be due to ascertainment
strengths
Strengths
  • Large population-based study
  • Less susceptible to selection bias
  • Power
  • Diagnoses usually made with skin biopsy in secondary care
  • Previous studies confirm validity of diagnoses in THIN
  • Limited to “up to standard” data
limitations
Limitations
  • Routine data
  • Cannot comment on disease severity
  • Possible misclassification of prevalent cases; possible underestimation of mortality rates
conclusion
Conclusion
  • Increasing incidence rates over time
  • No variation by region or socioeconomic status
  • Mortality rates twice (pemphigoid) and three times (pemphigus) higher than matched controls
implications
Implications
  • Increasing population age, likely increase in morbidity and mortality from these diseases
  • Other measures of disease occurrence underestimate associated mortality
acknowledgements
Acknowledgements

Dr Chris Smith, Professor Liam

Smeeth, Dr Kate Fleming and

Professor Richard Hubbard

Professor Hywel Williams for guidance

and support