Diagnosis of Behçet syndrome based on clinical features

Bettiol, 2020Hatemi, 2018Hatemi, 2022Yazici, 2021

The diagnosis of Behçet syndrome is made on history and clinical examination. Refer patients to a specialist for confirmation of the diagnosis if Behçet syndrome is suspected. Internationally recognised criteria exist and a point-scoring system based on clinical features can be used to assist diagnosisInternational Team for the Revision of the International Criteria for Behcet's, 2014. In this point-scoring system, the presence of oral ulceration, genital ulceration and ocular inflammation each score 2 points, whereas skin lesions, neurological features, vascular features and a positive pathergy test each score 1 point. A patient who scores 4 or more points is classified as having Behçet syndrome.

The most common clinical features of Behçet syndrome include:

• recurrent painful oral ulceration (aphthae)
• recurrent painful genital ulceration (aphthae)
• ocular inflammation, particularly severe uveitis or retinal vasculitis
• cutaneous lesions characteristic of Behçet syndrome (eg acneiform lesions, erythema nodosum)
• neurological disease
• vascular features—arteriovenous thrombosis, vasculitis, phlebitis, aneurysms
• positive pathergy test, noting that this is uncommon in the Australian patient population
• mono- or oligoarthritis
• gastrointestinal disease.

See the sections on each clinical feature below for more details.