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

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. Access through your institution Buy or subscribe

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AN INTEGRATED CLINICAL AND MOLECULAR STUDY OF A COHORT OF TURKISH PATIENTS WITH MARFAN SYNDROME HARBORING KNOWN AND NOVEL _FBN1_ VARIANTS Article 22 January 2021 REVISED DIAGNOSTIC CRITERIA

FOR NEUROFIBROMATOSIS TYPE 1 AND LEGIUS SYNDROME: AN INTERNATIONAL CONSENSUS RECOMMENDATION Article Open access 19 May 2021 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

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Download references ACKNOWLEDGEMENTS The authors thank H. Direskeneli (Marmara University, Turkey) for his valuable comments during manuscript preparation. AUTHOR INFORMATION AUTHORS AND

AFFILIATIONS * Academic Hospital, Internal Medicine (Rheumatology), Nuhkuyusu cad. Uskudar, Istanbul, 34668, Turkey Hasan Yazici * Department of Internal Medicine, Division of Rheumatology,

University of Istanbul, Cerrahpaşa Medical Faculty, 181 Kocamustafapaşa, Istanbul, 34098, Fatih, Turkey Emire Seyahi & Gulen Hatemi * Department of Medicine (Rheumatology), New York

University School of Medicine, NYU Hospital for Joint Diseases, 333 East 38th Street, New York, 10016, NY, USA Yusuf Yazici Authors * Hasan Yazici View author publications You can also

search for this author inPubMed Google Scholar * Emire Seyahi View author publications You can also search for this author inPubMed Google Scholar * Gulen Hatemi View author publications You

can also search for this author inPubMed Google Scholar * Yusuf Yazici View author publications You can also search for this author inPubMed Google Scholar CONTRIBUTIONS 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. CORRESPONDING AUTHOR

Correspondence to Hasan Yazici. ETHICS DECLARATIONS COMPETING INTERESTS 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. POWERPOINT SLIDES POWERPOINT SLIDE FOR FIG. 1 POWERPOINT SLIDE FOR FIG. 2 POWERPOINT SLIDE FOR FIG. 3

POWERPOINT SLIDE FOR FIG. 4 POWERPOINT SLIDE FOR TABLE 1 POWERPOINT SLIDE FOR TABLE 2 RIGHTS AND PERMISSIONS Reprints and permissions ABOUT THIS ARTICLE CITE THIS ARTICLE 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 Download citation * Published: 03 January 2018

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