VALIDATION OF THE INTERNATIONAL STUDY GROUP CRITERIA FOR BEHÇET'S DISEASE

AbstractObjective:To investigate nasal mucociliary clearance in patients with Behçet's disease.Methods:Thirty patients with Behçet's disease, diagnosed on the basis of criteria defined by the International Study Group for Behçet's Disease, and 30 healthy individuals were recruited. In both groups, individuals with conditions that could affect nasal mucociliary clearance were excluded. Nasal mucociliary clearance time was evaluated using saccharin tests. The time between placement of a saccharin tablet and the tasting of the saccharin was measured and recorded as the saccharin clearance time.Results:The mean nasal mucociliary clearance time was significantly longer in the Behçet's disease patients than in the control subjects (p < 0.001). Furthermore, there was a positive correlation between Behçet's disease duration and nasal mucociliary clearance time (p < 0.001, r = 0.882).Conclusion:The results suggest that nasal mucociliary clearance time is longer in Behçet's disease patients than in control subjects, and is positively correlated with disease duration.

Download Full-text

Behçet's Disease from Rheumatology and Neurology Perspectives with Special Reference to Children

Journal of Pediatric Neurology ◽

10.1055/s-0039-1692451 ◽

2019 ◽

Vol 18 (04) ◽

pp. 175-181

Author(s):

Yaşar B. Turgut ◽

Ayşe F. Tosun ◽

Beste K. Yüzbaşı ◽

Hasan Tunca

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

International Study ◽

Skin Lesions ◽

Disease Classification ◽

Behcet's Disease ◽

Male Predominance ◽

Pediatric Age Group ◽

Pathergy Test

AbstractBehçet's disease (BD) is an idiopathic chronic relapsing multisystemic vascular inflammatory disease. Although it is generally seen in young adults with male predominance, the onset age of the disease is the pediatric age group in only 4 to 26% of all cases of BD. According to the International Study Group for Behçet's Disease Classification Criteria, a diagnosis of BD requires recurrent oral aphthous ulcerations with two of the following: genital ulcerations, skin lesions, eye lesions, or a positive pathergy test. In this article, we extensively reviewed the current literature with respect to the rheumatological and neurological findings of BD in childhood.

Download Full-text

Pediatric Behçet’s Disease and Thromboses

The Journal of Rheumatology ◽

10.3899/jrheum.100257 ◽

2010 ◽

Vol 38 (2) ◽

pp. 387-390 ◽

Cited By ~ 23

Author(s):

BRICE KRUPA ◽

ROLANDO CIMAZ ◽

SEZA OZEN ◽

MICHEL FISCHBACH ◽

PIERRE COCHAT ◽

...

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

International Study ◽

Retrospective Chart Review ◽

Lower Limbs ◽

Disease Course ◽

Protein C Deficiency ◽

Behcet's Disease ◽

Increased Risk

Objective.To describe the characteristics of a group of pediatric patients with Behçet’s disease (BD) who presented at least 1 episode of thrombosis during their disease course.Methods.We made a retrospective chart review of the clinical, biological, and radiological data of children with BD who presented at least 1 episode of either arterial or venous thrombosis. Data were extracted from both an international pediatric Behçet cohort and files referred from 7 French centers.Results.Twenty-one patients were included. Diagnosis of BD was based on the criteria of the International Study Group for BD. Main locations for thrombosis were the cerebral sinuses, in 11 patients (52.4%); and lower limbs, in 9 patients (40.9%). Recurrent episodes were observed in 4 patients (21%). Thrombophilia measurements were normal in 14 patients out of 21, while anticardiolipin antibodies were positive in 4 patients, and 2 out of 21 had protein C deficiency. One patient had lupus anticoagulant. All patients were treated with colchicine. Corticosteroids were also added for variable periods in 13 patients. Five patients out of 21 were treated with anticoagulants (heparin, then anti-vitamin K) and 3 with antiplatelets (acetylsalicylic acid).Conclusion.Thromboses are a serious complication of BD and may occur early in the disease course. The presence of thrombophilic markers could increase the risk of thrombosis in BD, but the size of our population does not allow any conclusion. An international cohort (PED-BD) is currently in place and will allow study of such cases longitudinally, as well as assessment of the elements that correlate with an increased risk of thrombosis in children with BD.

Download Full-text

Prognostic analysis of Behçet’s disease with aortic regurgitation or involvement

Netherlands Heart Journal ◽

10.1007/s12471-021-01567-6 ◽

2021 ◽

Author(s):

X. Li ◽

X. Wen ◽

J. Xu ◽

Q. Lin ◽

L. Liu

Keyword(s):

Surgical Treatment ◽

Aortic Regurgitation ◽

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

International Study ◽

Aortic Disease ◽

Chinese Patients ◽

Behcet's Disease ◽

Significant Difference

Abstract Background Aortic regurgitation is the most common cardiovascular damage in Chinese patients with Behçet’s disease (BD) and is usually associated with aortic disease. These patients are easily misdiagnosed, and their prognosis is poor, even after surgical treatment. This study aimed to analyse potential factors that can improve the prognosis of BD patients with aortic regurgitation and/or aortic involvement. Methods Twenty-two patients with diagnosed or suspected BD as well as aortic regurgitation and/or aortic involvement in our hospital from 2012 through 2017 were collected in this study. Their clinical characteristics were listed, and the diagnosis of BD was evaluated by two different criteria sets. The influences of surgical treatment and immunosuppressive therapy (IST) on their prognosis were also explored. Results The diagnostic positive rate of the International Criteria for Behçet’s Disease was higher than that of the International Study Group criteria (kappa value 0.31, p < 0.05), indicating that the diagnostic consistency between the criteria sets was poor. There was no significant difference in survival between patients who had undergone ≤ 1 operation and those with ≥ 2 operations. Aortic valve replacement alone or in combination with aortic root replacement had no significant effect on the incidence of reoperation or death, but IST did significantly reduce this incidence (p < 0.05). However, there was no significant difference in the occurrence of reoperation or death between preoperative and postoperative IST versus postoperative IST only. Conclusion IST significantly improved the prognosis of BD patients with aortic regurgitation and/or aortic involvement.

Download Full-text