Behçet syndrome: a contemporary view

A Corrigendum to this article was published on 24 January 2018

This article has been updated

Key Points

  • Both the disease presentation and evidence from basic studies suggest more than one pathogenetic mechanism is involved in Behçet syndrome. Recognized vascular manifestations in Behçet syndrome include venous claudication, bronchial arterial collaterals (causing haemoptysis) and 'silent' Budd–Chiari syndrome

  • The diagnostic specificity of certain manifestations, such as eye disease or vascular involvement, might be more pathognomonic than other manifestations, such as gastrointestinal ulcerations

  • In considering the clinical and the basic science findings in Behçet syndrome, the weight of evidence suggests Behçet syndrome should not to be grouped with other, seemingly related, conditions

Abstract

The presence of symptom clusters, regional differences in disease expression and similarities with, for example, Crohn's disease suggest multiple pathological pathways are involved in Behçet syndrome. These features also make formulating disease criteria difficult. Genetic studies have identified HLA-B*51 to be the important genetic risk factor. However, the low prevalence of HLA-B*51 in many patients with bone fide disease, especially in non-endemic regions, suggests other factors must also be operative in Behçet syndrome. This consideration is also true for the newly proposed 'MHC-I-opathy' concept. Despite lacking a clear aetiological mechanism and definition, management of manifestations that include major vascular disease (such as Budd–Chiari syndrome and pulmonary artery involvement), eye disease and central nervous system involvement has improved with the help of new technology. Furthermore, even with our incomplete understanding of disease mechanisms, the prognoses of patients with Behçet syndrome, including those with eye disease, continue to improve.

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Figure 1: The prevalence of Behçet syndrome.
Figure 2: Vascular involvement in Behçet syndrome.
Figure 3: CNS involvement in Behçet syndrome.
Figure 4: Management of Behçet syndrome.

Change history

  • 24 January 2018

    In the original version of this article, the indicated dosage of colchicine, 1.5 mg per day, was incorrectly given as 1.5 mg/kg per day in figure 4. This error has now been corrected in the print, PDF and HTML versions of this article.

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Acknowledgements

The authors thank H. Direskeneli (Marmara University, Turkey) for his valuable comments during manuscript preparation.

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All authors researched the data for the article, contributed substantially to the discussions of its content, wrote the manuscript and reviewed the manuscript before submission.

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Correspondence to Hasan Yazici.

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E.S. has received honoraria or speaker's fees from Pfizer, MSD, Mustafa Nevzat and UCB Pharma. G.H. has received honoraria, speaker fees and/or research grants from Abbvie, BMS, Celgene, Mustafa Nevzat, MSD, Pfizer and UCB Pharma. Y.Y. has received research grants from BMS, Celgene and Genentech, and has received consulting fees from Celgene. H.Y. declares no competing interests.

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Yazici, H., Seyahi, E., Hatemi, G. et al. Behçet syndrome: a contemporary view. Nat Rev Rheumatol 14, 107–119 (2018). https://doi.org/10.1038/nrrheum.2017.208

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