ABSTRACT

Objective:

Behçet’s syndrome (BS) is a systemic vasculitis that can affect both veins and arteries. In this study, we aimed to review the demographic and clinical characteristics, treatments of patients who were followed up with the diagnosis of BS in our newly established clinic, and mainly focus on vascular BS cases.

Methods:

The data of 127 patients who applied between July 2020 and December 2022 and were followed up with the diagnosis of BS were retrospectively reviewed. Of the patients, 78 (61%) were male, 49 (39%) were female. There was vascular involvement in 49 (39%) patients.

Results:

Three of them had isolated dural sinus thrombosis and these patients were excluded. Of 46 patients, 37 (80.4%) had lower extremity vein thrombosis, 12 (26%) had pulmonary artery involvement. Three patients had coronary artery involvement and four had intracardiac thrombus. In addition to glucocorticoids, azathioprine was the first preferred immunosuppressive agent in patients with venous thrombosis. Until 2022, our treatment choice in pulmonary artery involvement was cyclophosphamide followed by azathioprine or infliximab maintenance. In 2022, treatment was started directly with the infliximab and azathioprine in 4 patients with pulmonary artery involvement. In patients with coronary artery involvement, treatment was started with cyclophosphamide. Two of the patients had died and both were male (central nervous system in one, coronary artery and pulmonary artery involvement in the other).

Conclusion:

Vascular involvement continues to be an important cause of morbidity and mortality in BS. Early recognition and treatment of these patients is important.

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Behçet’s syndrome and vascular involvement in a new rhematology clinic

ABSTRACT

References