2Behçet's syndrome
Introduction
In 1937, Hulusi Behçet described three patients with a triple symptom complex of aphthae, genital ulcers, and hypopyon uveitis.1 Subsequent studies showed this entity to be a multisystem disease characterized by variable clinical manifestations. Almost all patients have recurrent oral aphthae, followed in frequency by genital ulcers, variable skin lesions, arthritis, uveitis, thromboplebitis, and gastrointestinal and central nervous system involvement. Behçet's syndrome (BS) is a systemic vasculitis of small and large vessels, of both the venous and arterial side, with an unknown aetiology.2, 3
This review of BS focuses on recent developments in the current understanding of the epidemiology, describing the major vessel involvement and the diagnostic criteria, and addressing the evidence-based therapies, as well as new approaches such as biological agents in the treatment.
Section snippets
Epidemiology
BS is most commonly seen in those countries that extend from the Mediterranean to the Far East along the ancient trading route known as the ‘silk road’. This suggests that the putative agent(s), including several genetic factors such as HLA-B51, spread along this route.4
The prevalence of BS was between 20 and 421 per 100,000 adults in five field surveys carried out in Turkey (Table 1)5, 6, 7, 8, 9; there was not a single patient among 47,000 children in another field survey from the same
Clinical manifestations
Mucocutaneous features are the most common presenting symptoms of the disease; eye, vascular and neurological involvement are the most serious.
Pathology and pathogenesis
Although vascular injury is quite common and can involve all sizes of vessels, both on the venous and the arterial side, frank necrotizing vasculitis of the small and medium-sized arteries of the type seen in anticardiolipin and antineutrophilic antibodies (ANCA)-associated vasculitides is infrequent. Similarly infrequent are giant cells and immune-complex-type cutaneous venulitis. These make the vascular involvement in BS rather unique.54 Furthermore, there is little evidence for vasculitis in
Laboratory investigations
There are no specific laboratory findings. A moderate anaemia of chronic disease and leucocytosis is found in around 15% of patients. The erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) are usually moderately elevated but do not correlate well with disease activity.72 Serum immunoglobulins are sometimes elevated; complement levels might also be high. Autoantibodies such as rheumatoid factor (RF), antinuclear antibody (ANA), anticardiolipin and antineutrophilic antibodies
Diagnosis and differential diagnosis
As there are no specific symptoms and signs, the diagnosis of BS is based on the recognition of a group of clinical features. In 1990, the International Study Group published a set of diagnostic (classification) criteria based on data from a computer analysis of clinical features in 914 patients with BS and 308 diseased controls with features that can be confused with Behçet's syndrome from seven countries around the world.73 As these new criteria, which had a sensitivity of 91% and specificity
Prognosis and management
With time, BS goes into a complete remission in many patients.81 Younger patients and men generally have more severe disease, demonstrating an increased frequency both of mortality and of morbidity related to eye, vascular and neurological disease than women and older patients. Currently, loss of useful vision is seen in less than 10–15% of patients with eye involvement, compared with 75% of such patients 20–30 years ago. Whereas many patients with BS, especially older females, can be managed
Established (conventional) therapies
Recently, the European League Against Rheumatism (EULAR) has published guidelines for managing BS.98 These provide good guidance for the management of BS in organ systems other than the vascular, neurological and gastrointestinal systems (there is a lack of controlled studies relating to these pathologies). Topical measures, including local steroid application, are sufficient for isolated oral and genital ulcers.
As the main pathology is inflammation of the vessel wall and lack of
Conflict of interest
None.
Role of the funding source
None.
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