Clinical Pearls & Morning Reports

Posted by Carla Rothaus, MD

Published February 14, 2024


Are vascular complications common in Behҫet’s syndrome?

Behҫet’s syndrome is a chronic, multisystem, inflammatory condition with a relapsing and remitting course. Read the NEJM Review Article here.

Clinical Pearls

Q: Describe some of the epidemiological features of Behҫet’s syndrome.

A: The highest prevalence of Behҫet’s syndrome is found in Turkey, with 420 cases per 100,000 persons. The mean age at diagnosis is approximately 30 years, with the majority of patients presenting between the ages of 15 and 45 years. Disease activity tends to wane with advancing age. Although there is no difference in the incidence of Behҫet’s syndrome according to sex, male patients are more likely than female patients to have severe forms of the disease.

Q: Is the eye frequently affected in Behҫet’s syndrome?

A: The eye is the most frequently affected major organ in Behҫet’s syndrome, with ocular involvement occurring in approximately 50% of patients. Bilateral involvement is observed in 75 to 80% of new cases with ocular manifestations, on average occurring 2 years after the onset of the initial symptoms. Panuveitis is the most common presentation.

Morning Report Questions

Q: What is the most common clinical manifestation of Behҫet’s syndrome?

A: Recurrent oral ulceration, a hallmark of Behҫet’s syndrome, is the most common clinical manifestation, followed by genital ulcers, papulopustular lesions, and nodular skin lesions. Up to a third of patients present with only these manifestations throughout the course of their illness. Oral ulcers are typically both the initial and most persistent symptom in Behҫet’s syndrome. Roughly half the patients with Behҫet’s syndrome have joint involvement, which typically manifests as nondeforming, self-limited monoarthralgia or oligoarthralgia or arthritis, primarily affecting the knees, ankles, wrists, and elbows. The prevalence of gastrointestinal involvement varies substantially across ethnic groups, with rates below 5% in Europe and the Middle East and up to 20% in East Asia. Gastrointestinal ulcerations may occur throughout the gastrointestinal tract, leading to a range of clinical manifestations from mild to severe, including abdominal pain, diarrhea, and gastrointestinal bleeding. These symptoms may be difficult to distinguish from those found in patients with inflammatory bowel diseases.

Q: Are vascular complications common in Behҫet’s syndrome?

A: Vascular Behҫet’s syndrome can affect both veins and arteries of varying calibers and typically follows a relapsing course. Overall, the estimated 5-year cumulative risk of recurrent vascular events among patients with Behҫet’s syndrome is close to 40%. Superficial thrombophlebitis and deep-vein thrombosis are the most common manifestation of vascular Behҫet’s syndrome, occurring in 15 to 40% of cases. In patients with deep-vein thrombosis, there is a predilection for lower limb involvement, which can lead to a severe post-thrombotic syndrome. Pulmonary embolism is rare. Thrombotic events have also been noted in several other locations, including portal or suprahepatic veins (Budd–Chiari syndrome), the superior or inferior vena cava, and cerebral sinuses. Patients with arterial manifestations of Behҫet’s syndrome primarily present with aneurysms but may also have thrombotic occlusion or stenosis. Involvement of the pulmonary artery, although rare, is highly specific for Behҫet’s syndrome. Vascular Behҫet’s syndrome is the leading cause of death among patients with Behҫet’s disease, which is mainly due to arterial aneurysms (e.g., aortic or pulmonary) and Budd–Chiari syndrome.

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