Birmingham Centre of Excellence for Behçet’s Disease

A new centre which will revolutionise the experience and access to treatment for people with a rare and complex disease has opened in Birmingham.

The Birmingham Centre of Excellence for Behçet’s Disease has opened at the Birmingham and Midland Eye Centre (BMEC), at City Hospital. People with Behçet’s disease can now be seen by consultants from different specialties on the same day, instead of having to attend different appointments. The core services involved in this approach include rheumatology, opthalmology and oral medicine, with additional input from specialities such as neurology, dermatology and gastroenterology.

Behçet’s disease is a rare multi system vaculitic condition that causes inflammation of the blood vessels, mainly on the venous side of the circulation.  The symptoms of Behçet’s disease include recurrent oral and genital ulcers and potentially blinding eye inflammation. The disorder may also cause various types of skin lesions, arthritis, bowel inflammation and meningitis. Behçet’s disease generally begins when patients are in their 20s or 30s, although all age groups may be affected. Behçet’s is a multisystem disease; it may involve all organs and affect the central nervous system, causing memory loss and impaired speech, balance and movement. The effects of the disease may include blindness, stroke, inflammation in the spinal cord and intestinal complications.

Doctors from the BMEC and the rheumatology department at City Hospital staff the clinic, as well as oral medicine doctors from Birmingham Dental Hospital. The clinic is currently held most weeks on Fridays from 8.45am to 1pm, though plans are in hand to extend the number of clinics offered as the service develops.

BMEC is one of only three centres in the country to offer a clinic of this kind. The centre was funded by NHS Specialised Services Commissioning following a successful bid from the Behçet’s Syndrome Society, in partnership with doctors specialising in the disease from centres in London, Birmingham and Liverpool.

Various schemes for diagnosis and classification have been suggested over the years, none of which are perfect, and there is no diagnostic blood test for the condition. Generally the international study group for BD classification criteria are used to help support diagnosis. The diagnosis in individual patients depends on the ability and experience of the physician in recognising the characteristic features of the condition and eliminating other possibilities, and depends on a high index of suspicion if the patient presents with the presence of oral ulcers (98% of patients) recurring at least three times a year, plus at least two of the following;

• Recurrent genital ulceration
• Eye lesions; anterior uveitis, posterior uveitis, cells on slit lamp exam in the anterior chamber Or Retinal vascultitis (opthalmologist observed)
• Skin lesions; erythema noduosum, pseudofolliculitis, papulopustular lesions

Or acneform nodules, positive pathergy test. Other combinations leading to suspicion of the diagnosis could be the presence of two or three possible manifestations such as:

• Painful recurrent mouth ulcers and genital ulcers
• Painful recurrent mouth ulcers and an inflamed eye
• Painful recurrent mouth ulcers, genital ulcers and an inflamed eye
• Painful recurrent mouth ulcers, genital ulcers and inflamed joints
• Painful recurrent mouth ulcers, genital ulcers and skin lesions
• Inflamed eye(s) and joints and skin manifestations
• Inflamed eye(s), thrombophlebitis and skin manifestations
• Painful recurrent mouth ulcers, an inflamed eye and a positive family history

For more information, contact Dr Deva Situnayake, clinical lead for the Birmingham Centre of Excellence for Behçet’s Disease (or one of his colleagues), on 0121 507 4243. Potential referrals can be discussed and guidance provided on the referral process. Alternatively, for enquiries and high cost drug requests on the basis of the Behcet’s disease drugs pathway, email behcetsbham@nhs.net, which is linked to the clinicians 24/7 with a 24 hour response time.