AB0764 MANIFESTATIONS OF BEHCET’S DISEASE
Authors: R. Goloeva, Z. Alekberova
Journal: Annals of the Rheumatic Diseases (2021)

Background: Behcet’s disease (BD) is a systemic vasculitis of unknown etiology, characterized by recurrent oral and genital erosions and ulcerations, as well as involvement of joints, ocular, vascular, gastrointestinal (GI), and central nervous system (CNS). Objectives: To study the influence of such factors as patient’s gender, age at the disease onset and initial manifestations of the disease on the severity of BD. Methods: 95 patients with confirmed BB were examined. The majority of patients were males (70 men, 25 women) with the 2.8:1 M:W ratio. Patients’ mean age (median [interquartile range]) was 29 years [22; 34], from 16 to 51 years, the average age at disease onset was 20 years [14; 24]; and mean disease duration was 8 years [4; 15]. Results: The following clinical manifestations of BD were documented at disease onset (%): recurrent aphthous stomatitis – 63.1; skin lesions – 12.6; genital ulcers – 4.2; ocular lesions – 6.3; joint syndrome -7.4; CNS lesions -2.1; gastrointestinal tract involvement -2.1; thrombosis -1; epididymitis -1. 11.6% of all patients had signs of organ system involvement by the time of establishing BD diagnosis. Recurrent aphthous stomatitis was more common in female patients at BD onset– 76% vs. 58.6%, p =0.01, while organ system involvement was more common in males-14% vs. 4% in females, p =0.02. Progression from initial symptoms to the mature BD phenotype allowing to verify the diagnosis occurred within 4.3 years on average [1; 6], although 30.5% of patients progressed to a full-blown disease within 1 year. Analysis of potential predisposing or precipitating factors prior to BD onset identified such in only 12 patients, including frequent episodes of tonsilitis in 3 patients, cold exposure with hypothermia in 2, dental procedures in 2, stressful events - in 2, and one case of each - herpes labialis, frontal sinus puncture and appendectomy. Other patients could not recall any potential precipitator of BD. Mean age at BD onset was 20 years [14; 24], including 30.5% of patients with disease onset before 16 years, 22.1% aged from 16 to 21 years, 36.8% aged from 21 to 30 years at disease onset, and in remaining 10.5% the disease manifested after 30 years of age. BD onset in men and women was documented at approximately the same time. There were no signs or correlations indicating that patient’s age at disease onset could be associated with specific clinical symptoms in males or females. However, analysis of gender-associated differences in disease severity based on Krause’s Clinical Severity Scoring yielded the following results: mild BD was found in 15.7% men and 44% women ( p =0.009), moderate disease - in 7.1% men and 16% women, ( p &gt;0.05), and severe BD - in 77% men and 40% women, respectively, ( p =0.001). The risk of developing severe BD was 5-fold higher in men vs women [OR = 5.06, 95% CI 1.7-15.1]. Conclusion: First BD symptoms manifested in the majority of patients before the age of 30 years, with only 10% of patients with BD onset at the age of &gt; 30 years. In more than 50% of all patients aphthous stomatitis was the first manifestation of the disease, and was more common in women. Prognostically unfavorable BD signs were found in 11.6% of patients already at the initial stages of the disease in men. No correlation was found between the age at BD onset and clinical symptoms of the disease in both males and females. Disclosure of Interests: None declared