Behcet's disease (BD) is a multisystemic inflammatory disorder known as having a histopathological findings of vasculitis. The influence of sexual difference on BD is a well-known fact and there are several reports suggesting a more severe course of the disease among young males. The purpose of our study was to determine the effects of gender on the severity and clinical features of BD patients in Korea. The study included 1,901 patients with BD who fulfilled the criteria of International Study Group for Behcet's Disease or corresponded to the complete or incomplete type for the revised criteria of Behcet's Disease Research Committee of Japan. BD in Korea showed a female predominance (M:F=0.61:1). The skin lesions were observed in 79.9% of patients, of which 77.6% had erythema nodosum-like lesion, which was more frequent in females. The ocular lesions were more common in males showing a higher frequency of uveitis. Ocular and vascular symptoms as clinical features with severe complications or mortality were more frequent in males than in females. The mean age at the onset of patients with the worst prognosis such as ocular, gastrointestinal, neurologic, and vascular involvements was significantly younger in male than in female patients (p<0.05). In conclusion, this study elucidated the influences of sexual difference on BD in Korea.
Adolescent ; Adult ; Age of Onset ; Aged ; Behcet Syndrome/epidemiology ; Behcet Syndrome/physiopathology* ; Child ; Female ; Human ; Korea ; Male ; Middle Aged ; Retrospective Studies ; Sex Characteristics*

Behcet's disease is a multisystem disorder that affects mainly young adults. Conflicting reports of the effects of pregnancy on the course of Behcet's disease have been reported. We studied 27 pregnant women with Behcet's disease using questionnaires, clinical examination, and laboratory tests. The condition of 18 patients worsened (66.7%) and 9 patients (33.3%) improved during pregnancy. In the deteriorated group, clinical exacerbation of Behcet's disease occurred most commonly during the first trimester (77.8%). They were primarily of the suspected or mucocutaneous type, while in the improved group, the majority of patients were of the incomplete and complete type, or the arthritic and ocular type. Of interest was the fact that all patients in the improved group showed exacerbations related to menstruation or postpartum, which indicated that progesterone may be the major hormone influencing the course of Behcet's disease.
Adult ; Behcet's Syndrome/physiopathology* ; C-Reactive Protein/analysis ; Female ; Human ; Infant, Newborn ; Menstrual Cycle ; Pregnancy ; Pregnancy Complications/physiopathology*

The authors present the clinical findings of a 30-year-old female and a 29-year-old male who both had isolated unilateral lateral rectus muscle palsy in neuro-Behcet's disease. The clinical feature related to isolated abduscens nerve palsy was identified by CT, systemic assessment and extraocular examination. These patients' constellation of findings appear to be unique: it does not follow any previously reported pattern of ocular manifestations of neuro-Behcet's disease.
Abducens Nerve/physiopathology ; Adult ; Behcet's Syndrome/complications* ; Case Report ; Eye Movements/physiology ; Female ; Human ; Male ; Ophthalmoplegia/physiopathology ; Ophthalmoplegia/etiology* ; Paralysis/physiopathology ; Paralysis/etiology*

We report the case of a 41-year-old Chinese woman with Behçet's disease (BD) complicated by acute myocardial infarction, requiring inotropic and ventilatory support. Angiography showed critical left anterior descending coronary artery stenosis, a blocked left subclavian artery and left carotid artery stenosis. The patient was successfully treated with a high dose of immunosuppressants, standard anti-ischaemic therapy and percutaneous coronary intervention. Although life-threatening, coronary arteritis is a treatable manifestation of BD. We suggest that the diagnosis of coronary arteritis be considered in patients with BD who present with chest pain. Involvement of other arteries should also be looked out for in these patients.
Adult ; Angiography ; Angioplasty, Balloon, Coronary ; Arterial Occlusive Diseases ; physiopathology ; Arteritis ; physiopathology ; Behcet Syndrome ; complications ; Coronary Angiography ; Coronary Vessels ; physiopathology ; Female ; Humans ; Infarction ; complications ; Inflammation ; physiopathology ; Magnetic Resonance Imaging ; Myocardial Infarction ; complications ; Prednisolone ; therapeutic use ; Subclavian Artery ; pathology ; physiopathology

PURPOSE: To identify significant fluorescein angiographic (FA) characteristics associated with visual acuity (VA) in Behcet retinal vasculitis. MATERIALS AND METHODS: Retrospective review of 86 eyes of 48 patients (age: 35.6+/-10.2 years) with Behcet retinal vasculitis were performed. VA and FA findings as well as correlation between them were assessed. RESULTS: The mean initial VA of eyes with posterior pole-involved vasculitis (63 eyes; 73.3%) was significantly worse than that of those with peripheral vasculitis (23 eye; 26.7%) (logarithm of the minimum angle of resolution VA: 0.554+/-0.572 vs. 0.078+/-0.148; p<0.0001). Subgroup analysis revealed a more severe and diffuse pattern of vascular leakage in posterior pole-involved vasculitis compared to peripheral vasculitis (p<0.0001). Retinal vascular leakage (beta=0.345; p<0.0001), optic disc hyperfluorescence (beta=0.147; p=0.032), and macular leakage (beta=0.107; p=0.047) were significantly associated with worse initial VA. During the follow up (mean: 33.3+/-17.9 months), the change of leakage showed no significant correlation with change of VA in posterior pole-involved vasculitis (tau=0.199, p=0.092). CONCLUSION: Posterior pole involvement, the degree of retinal vascular leakage, optic disc hyperfluorescence, and macular leakage are significantly associated with VA in Behcet retinal vasculitis.
Adult ; Aged ; Behcet Syndrome/*diagnosis ; Female ; *Fluorescein Angiography ; Fluoresceins ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Regression Analysis ; Retinal Vasculitis/*diagnosis/physiopathology ; Retrospective Studies ; Visual Acuity/*physiology

No abstract available.
Aneurysm, Dissecting/diagnosis/*etiology/physiopathology/therapy ; Anticoagulants/therapeutic use ; Aortic Aneurysm/diagnosis/*etiology/physiopathology/therapy ; Behcet Syndrome/*complications/diagnosis/drug therapy ; Cerebral Infarction/diagnosis/etiology ; Diffusion Magnetic Resonance Imaging ; Echocardiography, Doppler, Color ; Electrocardiography ; Hemodynamics ; Humans ; Immunosuppressive Agents/therapeutic use ; Male ; Middle Aged ; *Sinus of Valsalva/physiopathology/ultrasonography ; Thrombosis/diagnosis/drug therapy/*etiology/physiopathology ; Ventricular Septal Rupture/diagnosis/*etiology/physiopathology/therapy

Endovascular repair of inflammatory aortic aneurysms has been reported as an alternative to open surgical treatment. In selective cases, adjunctive bypass surgery may be required to provide an adequate landing zone. We report a case of endovascular repair of an inflammatory aortic aneurysm in a patient with Behcet's disease using a carotid-carotid bypass graft to provide an adequate landing zone. A 45-yr-old man with a voice change was referred to our hospital with the diagnosis of saccular aneurysm of the distal aortic arch resulting from vasculitis. Computed tomography showed a thoracic aortic aneurysm with thrombosis. Right to left carotid-carotid bypass grafting was performed. After 8 days, the patient underwent an endovascular stent graft placement distal to the origin of the innominate artery. The patient was discharged with medication and without postoperative complications after 5 days. Hybrid endovascular treatment may be suitable a complementary modality for repairing inflammatory aortic aneurysms.
Aortic Aneurysm, Thoracic/complications/radiography/*surgery ; Behcet Syndrome/*complications/surgery ; Blood Vessel Prosthesis Implantation/*methods ; Carotid Arteries/physiopathology/*surgery ; Coronary Artery Bypass ; Endovascular Procedures ; Humans ; Male ; Middle Aged ; Stents ; Thrombosis/complications ; Tomography Scanners, X-Ray Computed ; Vasculitis/complications

No abstract available.
Behcet Syndrome/*complications/diagnosis/drug therapy ; Drug Therapy, Combination ; Hemianopsia/diagnosis/*etiology/physiopathology ; Humans ; Immunosuppressive Agents/administration & dosage ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Pulse Therapy, Drug ; Steroids/administration & dosage ; Treatment Outcome ; Visual Field Tests ; Visual Fields

BACKGROUND/AIMS: This study was undertaken to perform a cross-cultural adaptation of the Behcet's Disease Current Activity Form (BDCAF, version 2006) questionnaire to the Korean language and to evaluate its reliability and validity in a population of Korean patients with Behcet's disease (BD). METHODS: A cross-cultural study was conducted among patients with BD who attended our rheumatology clinic between November 2012 and March 2013. There were 11 males and 35 females in the group. The mean age of the participants was 48.5 years and the mean disease duration was 6.4 years. The first BDCAF questionnaire was completed on arrival and the second assessment was performed 20 minutes later by a different physician. The test-retest reliability was analyzed by computing kappa statistics. Kappa scores of > 0.6 indicated a good agreement. To assess the validity, we compared the total BDCAF score with the patient's/clinician's perception of disease activity and the Korean version of the Behcet's Disease Quality of Life (BDQOL). RESULTS: For the test-retest reliability, good agreements were achieved on items such as headache, oral/genital ulceration, erythema, skin pustules, arthralgia, nausea/vomiting/abdominal pain, and diarrhea with altered/frank blood per rectum. Moderate agreement was observed for eye and nervous system involvement. We achieved a fair agreement for arthritis and major vessel involvement. Significant correlations were obtained between the total BDCAF score with the BDQOL and the patient's/clinician's perception of disease activity p < 0.05). CONCLUSIONS: The Korean version of the BDCAF is a reliable and valid instrument for measuring current disease activity in Korean BD patients.
Adult ; Asian Continental Ancestry Group/psychology ; Behcet Syndrome/*diagnosis/physiopathology/psychology ; Comprehension ; Cultural Characteristics ; Female ; Humans ; Language ; Male ; Middle Aged ; Observer Variation ; Predictive Value of Tests ; Reproducibility of Results ; Republic of Korea/epidemiology ; Severity of Illness Index ; *Surveys and Questionnaires

Behcet's disease with concomitant thrombotic thrombocytopenic purpura (TTP), coronary artery stenosis and coronary artery pseudo aneurysm is rare. Here we report a case of Behcet's disease with several cardiovascular complications, namely: pericarditis, deep vein thrombosis (DVT), TTP, coronary artery stenosis, and a coronary artery pseudo aneurysm. A 37-year-old female presented with sudden dyspnea and syncope at our emergency room and underwent pericardiectomy and pericardial window formation for the diagnosis of cardiac tamponade with acute hemorrhagic pericarditis. Thereafter, TTP and DVT complicated her illness. After confirmation of Behcet's disease on the basis of a history of recurrent oral and genital ulcers and erythema nodosum, remission was achieved after treatment with methylprednisolone pulse therapy, colchicine, catheter directed thrombolysis and thrombectomy. However, whilst maintaining anticoagulation therapy, a newly developed pericardial aneurysmal dilatation was noted on follow-up radiologic evaluation. Further evaluation revealed right coronary artery stenosis and a left coronary artery pseudo aneurysm; these additional problems were treated with the nonsurgical insertion of an endovascular graft stent . At the time of writing three months later after stent insertion, the aneurysm has continued to regress and no additional complications have intervened with combined immunosuppressive therapy.
Venous Thrombosis/diagnosis/*etiology ; Purpura, Thrombotic Thrombocytopenic/diagnosis/*etiology ; Pericarditis/diagnosis/*etiology ; Humans ; Female ; Echocardiography ; Coronary Vessels/*physiopathology ; Coronary Stenosis/diagnosis/*etiology ; Cardiac Tamponade/diagnosis/*etiology ; Behcet Syndrome/*complications/diagnosis ; Aneurysm, False/diagnosis/*etiology ; Adult