Autoimmune blistering diseases: Incidence, prevalence, co-morbidity and prescription patterns

Study type
Protocol
Date of Approval
Study reference ID
18_224
Lay Summary

There are two serious but rare blistering skin conditions called 'bullous pemphigoid' and 'pemphigus vulgaris'. They occur when the body does not recognise its own tissues and attacks them. This results in painful blisters and open sores. When the mouth is affected the extreme pain causes difficulty in eating and drinking. There is no cure only treatment that aims to control symptoms.

Steroid tablets are commonly used to control the symptoms but they can cause very serious side effects, including thinning bones and fractures, diabetes and infections. There is an urgent need to conduct high quality trials to find better treatments but, because the conditions are rare, we first need to establish how many patients there would be to take part in the trials and how they are currently treated.

We will use the CPRD to find out how many people would be available to take part in future trials.
This study will show how many people in the UK have developed bullous pemphigoid and pemphigus vulgaris in the last 20 years, the dose and length of time people are prescribed steroid tablets, any reasons why they could not take part in a trial.

Technical Summary

Bullous pemphigoid and pemphigus vulgaris are serious autoimmune blistering skin conditions associated with high risk of death and can last for several years. The most common treatment for these diseases is steroid tablets which control the symptoms, but can cause serious side effects including infections and diabetes. Recent research has shown alternative treatment may be effective and safe. Further trials are required but before developing them it is necessary to know the number of eligible patients and what standard care is, in the UK. We will use the CPRD and linked HES to determine the prevalence, incidence, prescription patterns and number of eligible patients to be conducted in future trials on BP and PV. We will use Poisson regression modelling to determine adjusted incidence rate ratios by calendar year, age group, gender, geographical region and socioeconomic group. We will also determine the proportion of misclassification of diagnostic codes to determine the most appropriate codes to use to identify patients with these conditions.

Health Outcomes to be Measured

 The reliability of diagnostic codes to identify people with pemphigoid and pemphigus vulgaris
- The prevalence and incidence of bullous pemphigoid and pemphigus vulgaris
- The prevalence of specific co-morbidity in people with bullous pemphigoid and pemphigus vulgaris
- Oral prednisolone use in people with bullous pemphigoid and pemphigus vulgaris

Collaborators

Sonia Gran - Chief Investigator - University of Nottingham
Sonia Gran - Corresponding Applicant - University of Nottingham
Joanne Chalmers - Collaborator - University of Nottingham
Julia Hippisley-Cox - Collaborator - University of Oxford
KAREN HARMAN - Collaborator - University of Nottingham
Kim Thomas - Collaborator - University of Nottingham
Sinead Langan - Collaborator - London School of Hygiene & Tropical Medicine ( LSHTM )

Linkages

HES Admitted Patient Care;ONS Death Registration Data;Patient Level Index of Multiple Deprivation;Practice Level Index of Multiple Deprivation