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  • Review Article
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Differential diagnosis and management of Behçet syndrome

Nature Reviews Rheumatology volume 9, pages 79–89 (2013)Cite this article

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Abstract

Behçet syndrome (also known as Behçet disease) is a rare condition that is associated with considerable morbidity. Cases of Behçet syndrome have been reported worldwide, but the highest prevalence occurs in countries that border the ancient Silk Route, such as Turkey and Iran. Although oral ulceration, genital ulceration and eye disease are the classic triad of manifestations, the cardiovascular, gastrointestinal, musculoskeletal and central nervous systems can also be affected. The syndrome is chronic and relapsing with some patients having benign episodes whereas others have more serious complications, including blindness or the rupture of a pulmonary arterial aneurysm. Diagnosing Behçet syndrome, particularly outside of endemic regions, often incurs a considerable delay owing to the rarity of this condition. Furthermore, a paucity exists of data from randomized controlled trials on the optimal therapeutic approaches to use in patients, as well as a lack of informative laboratory surrogate markers to monitor disease progression. This Review discusses the issues surrounding the diagnosis and differential diagnosis of Behçet syndrome and presents the current approaches to managing patients with this complex group of disorders.

Key Points

  • Behçet syndrome is a multisystem condition, the hallmark features being orogenital ulceration and eye disease (typically bilateral panuveitis and retinal vasculitis)

  • Behçet syndrome commonly affects the skin, and additionally the cardiovascular, neurological, gastrointestinal and musculoskeletal systems may all be involved

  • Severity of clinical manifestations and mortality risk are generally greatest in men, the young (that is, those <35 years in age) and those with genetic links to endemic areas

  • No diagnostic test currently exists for Behçet syndrome, although the International Study Group diagnostic criteria can be useful for diagnosing patients

  • Limited data regarding the treatment of Behçet syndrome are available from randomized controlled trials

  • Treatment is based on immunosuppression with agents including steroids, azathioprine, and anti-TNF biologic agents

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Figure 1: CT image of a brain of a 40-year-old woman presenting with headaches, vomiting and dizziness.
Figure 2: Brain scan of a 40-year-old male with a longstanding history of Behçet syndrome.
Figure 3: Magnetic resonance angiogram of the iliac and femoral arteries.

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Acknowledgements

The authors have received funding from the British Heart Foundation and Arthritis Research UK, which has made this Review possible. The authors also gratefully acknowledge the support of the National Institute of Health Research Comprehensive Biomedical Research Centre at Imperial College Healthcare NHS Trust.

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  1. National Heart and Lung Institute, Imperial College London, Du Cane Road, W12 0NN, London, UK

    Nicola L. Ambrose & Dorian O. Haskard

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N. L. Ambrose researched data for the article. Both authors contributed to the discussion of content, writing and editing of the article.

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Correspondence to Dorian O. Haskard.

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Ambrose, N., Haskard, D. Differential diagnosis and management of Behçet syndrome. Nat Rev Rheumatol 9, 79–89 (2013). https://doi.org/10.1038/nrrheum.2012.156

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