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Institute of Dentistry - Faculty of Medicine and Dentistry

A pioneering ‘one-stop’ clinic for Behçet’s syndrome transforms patient care

Behçet’s treatment at the BCE

Professor Fortune’s holistic approach to patient care changes the lives of Behçet’s syndrome patients.

Behçet’s syndrome is a rare chronic condition that causes inflammation of the blood vessels and tissues. It affects an estimated 0.64 per 100,000 individuals in the UK.

It can cause severe mouth ulcers that can progress to affect any organ in the body with debilitating consequences: joint pain, skin lesions, swollen eye tissue (uveitis). It can lead to blindness or life-threatening complications such as strokes and blood clotting if the central nervous system or major blood vessels become affected. There is currently no cure for Behçet’s syndrome.

Professor Fortune’s research has revealed how challenging life is for many Behçet’s patients: their complex symptoms, typically poor patient experience and substandard care lead to a generally reduced quality of life. Patients can undergo multiple appointments in different facilities to find that their isolated symptoms are met with inaccurate diagnoses and poor results.

Revealing the mechanisms behind the syndrome

Research by Queen Mary has helped to reveal the mechanisms behind Behçet’s syndrome. Professor Fortune and her team at Queen Mary are part of the international group that completed the first Behçet’s genome-wide association study in 2010 — a method that scans genomic markers across many individuals to identify genetic variants associated with the condition.

The data confirmed the major role of the antigen HLA-B51 in susceptibility to Behçet’s syndrome, which subsequently led to the discovery of genetic interactions between this antigen (HLA-B51) and the enzyme ERAP1, which is associated with immune responses and genes that trigger inflammation.

An effective ‘one stop clinic’ for patients

Professor Fortune’s work has been transformative, driving the creation in 2012 of three Behçet’s Syndrome Centres of Excellence (BCE) in London, Birmingham and Liverpool. Each centre contains a multidisciplinary clinic (MDC).

The team at the Behçet's Centre of Excellence at the Royal London Hospital
The team at the Behçet's Centre of Excellence at the Royal London Hospital

The Centres act as a ‘one stop clinic’ for 900 patients from both the UK and overseas. The BCEs play a leading role in Behçet’s research and have provided Professor Fortune’s team with recorded clinical outcomes, patient samples, and evidence of how Behçet’s impacts patients’ psycho-social wellbeing, employment, and levels of fatigue. Standardised oral and genital clinical severity scores have also been developed and are now used internationally. 

The Centres have transformed patient care and achieved hugely positive clinical outcomes – for example reducing the prevalence of serious complications such as disability and visual impairment (the latter of which saw a 97 per cent reduction from 2014–2019).

Not only have the Centres made a great difference to patient care, they have also proved highly cost-effective, saving approximately £18,800 per patient a year.

A multidisciplinary approach

Professor Fortune has pioneered a multidisciplinary, holistic approach to patients at the MDCs. Patients are reviewed by experts from multiple clinical specialities – oral medicine, immunology, rheumatology, gastroenterology, ophthalmology and neurology, together with a clinical psychologist and non-medical support worker. All clinical outcomes are reviewed regularly by the MDCs.

Professor Fortune and the BCE have also produced an agreed ‘drug pathway’ of systemic therapy and biologics that is centrally funded and delivered locally to patients.

Until 2012, it took a patient in the UK on average 14 years to achieve a diagnosis ... As a result of Queen Mary research, most diagnoses are now achieved within a year of presenting to clinic.

The patient knows best

To maintain a high level of patient care and support, the London BCE works closely with patients and clinicians in hospitals and the community. As one patient says, “The service is holistic, and [the] continuity of staff and doctors who know each other individually on a personal basis... facilitates much better patient care”.

The adoption of a ‘patient knows best’ strategy has been successful, with impressive patient satisfaction scores of over 99 per cent.

Rapid diagnosis: from 14 years to just one

Until 2012, it took a patient on average 14 years to achieve a diagnosis for Behçet’s syndrome in the UK.

Data from 2019 shows that, as a result of Queen Mary research, most diagnoses are now achieved within a year.

Improving patients’ physical health

Professor Fortune and her team have also identified effective treatment for two of the syndrome’s symptoms: mouth ulcers and blood clotting.

Severe mouth ulcers are the most frequent symptom of Behçet’s syndrome. Professor Fortune’s team developed and introduced oral health training and a bespoke mouthwash, Triorasol. In a study comprising of 261 Behçet’s patients, Triorasol significantly reduced oral ulceration severity scores (OUSS), while also reducing other forms of scarring, and has thus significantly improved patients’ quality of life.

Behçet’s patients also suffer from recurrent clotting throughout the body – even when they have been treated with anticoagulants. Professor Fortune’s team discovered that clotting happens because of disease related endothelial and platelet activation, explaining why anticoagulants are ineffective.

As a result, treatment guidelines for blood vessel clotting were changed to use immunosuppressives (ie suppress inflammatory ‘flares’ and endothelial swelling) and an antithrombotic (which, rather than inhibiting particular coagulation pathways, instead reduce disease activity by immunosuppressives platelet activity and thrombi formation).

Improving psychosocial health

The BCE also record Behçet’s impact on the psychosocial health of patients (and vice versa, by assessing how increased anxiety is associated with inflammation). Every patient is scored using Generalised Anxiety Disorder, Patient Health, and Mental Wellbeing questionnaires each time they visit the clinic, to assess depression, anxiety and mental wellbeing and evaluate the need for any emergency intervention and support. One patient with five self-directed emergency appointments saw decreased disease activity within six months.

This drives home the value of psychological support in tandem with systemic medication that improves anxiety, depression and clinical outcomes. As a result of Queen Mary’s work with the BCE, patients now report reduced anxiety and depression.

Living with Behçet syndrome can leave patients feeling isolated. To alleviate this, the BCE work closely with the society Behçet’s UK to improve access to patient support groups. Society membership increased to 1,114 in 2019.

There are also seven new support groups across England to help patients find additional help, such as trained counsellors or help navigating the benefits system. In the London BCE, this approach saw 441 of 845 ‘very highly vulnerable’ patients able to reach such assistance at any given time.

Informing and optimising clinical guidelines and practice

Queen Mary research demonstrated that gender, age and disease severity affect the activation of platelets and white blood cells in Behçet’s syndrome. This directly drove the European League Against Rheumatism (EULAR) to update its guidelines in 2018, which in turn improved subsequent clinical practice.

Patients formerly treated with the anticoagulant warfarin are now treated with antithrombotic, immunosuppressive, anti-inflammatory (aspirin) and anti-platelet drugs.

A new model of care

The BCE model was validated by the EU Commission’s rigorous audit process at the London Centre in September 2018, receiving an excellence rating of 97.2 per cent for clinical service, research and education. Through her research and key role in establishing and leading the BCE, Professor Fortune has demonstrated that a ‘one drug regime fits all’ strategy for clinical management isn’t appropriate for Behçet’s. It is neither beneficial to the patient nor a cost-effective use of resources.

Instead, the MDCs have enabled the effective application of appropriate, bespoke treatment, improving care while reducing the risk of patients developing complications.

By pioneering this multidisciplinary, patient-focused model of care, Professor Fortune has made it possible for Behçet’s syndrome patients to benefit from an integrated programme of diagnosis and treatment – one that has notably improved both clinical outcomes and patients’ quality of life.

 

Video

Behçet’s Children’s Clinic at The Royal London Hospital

This video was made by a patient and her family, recounting her experience of living with Behçet’s and of receiving care from Professor Fortune and her team.

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