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Review
. 2017 Aug 14:10:309-319.
doi: 10.2147/JMDH.S93681. eCollection 2017.

Behçet's syndrome: providing integrated care

Sinem Nihal Esatoglu  1 , Zekayi Kutlubay  2 , Didar Ucar  3 , Ibrahim Hatemi  4 , Ugur Uygunoglu  5 , Aksel Siva  5 , Gulen Hatemi  1
Affiliations
Review

Behçet's syndrome: providing integrated care

Sinem Nihal Esatoglu et al. J Multidiscip Healthc. .

Abstract

Behçet's syndrome (BS) is a multisystem vasculitis that presents with a variety of mucocutaneous manifestations such as oral and genital ulcers, papulopustular lesions and erythema nodosum as well as ocular, vascular, gastrointestinal and nervous system involvement. Although it occurs worldwide, it is especially prevalent in the Far East and around the Mediterranean Sea. Male gender and younger age at disease onset are associated with a more severe disease course. The management of BS depends on the severity of symptoms. If untreated, morbidity and mortality are considerably high in patients with major organ involvement. Multidisciplinary patient care is essential for the management of BS, as it is for other multisystem diseases. Rheumatologists, dermatologists, ophthalmologists, neurologists, cardiovascular surgeons and gastroenterologists are members of the multidisciplinary team. In this study, we reviewed the epidemiology, etiology, diagnostic criteria sets, clinical findings and treatment of BS and highlighted the importance of the multidisciplinary team in the management of BS.

Keywords: Behçet disease; diagnosis; epidemiology; management; multidisciplinary care; treatment; vasculitis.

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Conflict of interest statement

Disclosure Gulen Hatemi has received research grants, consulting fees or speaking fees from Abbvie, BMS, Celgene, MSD Pharmaceuticals, Mustafa Nevzat, UCB Pharma and Pfizer. The other authors report no conflicts of interest in this work.

Figures

Figure 1
Figure 1
Mucocutaneous lesions of Behçet’s syndrome. Notes: (A) OU; (B) GU with a scar; (C) PPL; (D) acne with arthritis; (E) EN and (F) leg ulcers. Abbreviations: OU, oral ulcer; GU, genital ulcer; PPL, papulopustular lesions; EN, erythema nodosum.
Figure 2
Figure 2
(A, B) Axial FLAIR-T2 images reveal hyperintense lesion in the brainstem (arrow), (C) axial T1 gadolinium sequence shows gadolinium enhancement (arrow) and (D) prominent brainstem atrophy (arrow) on the follow-up MRI after 3 years.
Figure 3
Figure 3
Endoscopic appearance of round ulcers of GI involvement of BS. Notes: (A, B) Some of these ulcers can be deep with elevated edges and (C, D) some are shallow. Abbreviations: GI, gastrointestinal involvement; BS, Behçet’s syndrome.
See this image and copyright information in PMC

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