The criteria employed for Korean Behcet's disease (BD) from January 1990 to December 2000 have been investigated, and the diagnostic validity for those criteria was determined. For the generation of a modified set of preliminary criteria from the International Study Group (ISG) criteria, the diagnostic values for individual feature of BD were calculated. The criteria by the Behcet's Disease Research Committee of Japan appeared to be widely employed with the ISG criteria. However, because the ISG criteria revealed a relatively valid outcome in Korea, the application of this criteria will be needed for the universal unification until the criteria with better performance comes out. On the other hand, the modified set of preliminary criteria that consisted of the clinical items with better results seemed to improve some pitfalls of the ISG criteria. Although that criteria showed better performance than the preexisting criteria, it should be necessary to validate its effectiveness in other areas.
Behcet Syndrome/classification ; Behcet Syndrome/diagnosis* ; Behcet Syndrome/epidemiology ; Bibliometrics ; Data Collection ; Databases, Factual ; Human ; Korea/epidemiology ; Likelihood Functions ; Prevalence ; Sensitivity and Specificity

Behcet's disease occurs with a high prevalence in the Far East including Korea. In this report we inspected 1,155 patients with Behcet's disease and collected information concerning the clinical and epidemiologic features of Behcet's disease in Korea. In summary, patients in their 30s were the most common, with the most common age of onset in the 20s; the sex ratio was 0.63:1 with female predominance; according to revised Shimizu's classification, the order of frequency of the different types was incomplete (38.2%), suspected (25.4%), possible (20.3%) and complete (16.1%); oral ulcers were the most frequent major symptom (97.5%) and the most common initial symptom (78.5%); erythema nodosum-like lesion (55.3%) was the most common type of skin lesion; a seasonal aggravation of the disease was observed in 21.3% of patients; and the most common type of childhood onset Behcet's disease was suspected type.
Adolescence ; Adult ; Age Distribution ; Aged ; Behcet's Syndrome/epidemiology* ; Behcet's Syndrome/complications ; Behcet's Syndrome/classification ; Child ; Female ; Human ; Korea/epidemiology ; Male ; Middle Age ; Sex Distribution

PURPOSE: Behcet's disease (BD) is a chronic, relapsing, multi-system vasculitis of unknown aetiology with complicated and diversified clinical features predominantly involving oral and genital ulcers, and ocular and cutaneous lesions. The clinical features of this disease have been described to be different according to geographical areas and gender. We investigated the specific clinical features of BD patients in Northeastern China. MATERIALS AND METHODS: 116 patients involved in this study fulfilled the classification criteria of the International Study Group for BD. The clinical manifestations and results of laboratory tests of BD were recorded in each patient. RESULTS: The onset was typically between 20-39 years with a slight female predominance. Oral ulcers were the most common manifestation, followed by skin lesions, positive pathergy reaction/genital ulcers, and ocular lesions. Vascular lesion and epididymitis were rare in patients with BD. The frequency of erythema nodosum-like lesion and articular involvement were significantly higher in females, while gastrointestinal involvement was significantly higher in males. The results of laboratory tests showed that the human leukocyte antigen (HLA)-B*51 alleles were positive in 30.9% of patients and the immunological abnormities were present in some patients. CONCLUSION: The clinical features of BD showed geographical and gender difference. Genetic and immune factors might participate in aetiopathogenesis of BD.
Behcet Syndrome/*diagnosis/epidemiology/pathology ; China ; Female ; Geography ; Humans ; Male

The German Registry of Adamantiades-Behcet's disease was founded in 1990 in Berlin and it provides current data on the epidemiology, the clinical manifestations and the course of the disease in Germany on a continuous basis. A total of 218 patients, including 89 German and 100 Turkish patients, had been reported to the German Registry until October 1997. One hundred and ninety-six patients fulfilled the criteria of the Behcet's disease classification tree. The prevalence of the disease evaluated in Berlin-West was 1.68/100,000 in 1989 and had risen to 2.26/100,000 by 1994. The median age of onset was 25 years (range 5 to 66 years; German-Turks, ns). Juvenile disease was recorded in 6.9% of patients. The complete clinical picture according to the criteria of the International Study Group of Behcet's Disease developed in 15.5 months. The interval between onset of the disease and diagnosis was 35 months, which was significantly longer than the duration of the development of the complete clinical picture (p < 0.0001). The disease was diagnosed later in German (48.5 months) than in Turkish patients (25.5 months, p = 0.003). While German patients presented an equal male-to-female ratio, a male predominance was shown in Turkish patients (M:F 2.1:1, p = 0.022). Familial occurrence was detected in 2.0% of German and 15.9% of Turkish patients (p = 0.013). The frequencies of major clinical manifestations were: oral ulcers 99%, skin lesions 76%, genital ulcers 75%, ocular manifestations 59%, arthritis 59%, and positive pathergy test 52%. Clinical differences between German and Turkish patients were only found in the frequency of ocular lesions (48% vs. 66%, p = 0.025). Oral ulcers were with 72% the most common onset symptom of the disease followed by erythema nodosum (9%), uveitis (7%), arthritis (7%), genital ulcers (3%), superficial thrombophlebitis (2%) and papules/sterile pustules (2%). Uveitis and erythema nodosum as onset symptoms shortened the median interval to diagnosis to 1.5 and 15 months, respectively, while arthritis delayed diagnosis (43.5 months; p = 0.029). A severe course developed in 25% of the patients; irreversible retinal vasculitis to blindness in 15%, sterile meningoencephalitis in 8%, severe arthritis in 5%, hemoptysis in 2%, lethal outcome in 2% and bowel perforation in 1%. The relative risk of HLA-B5 positive German natives developing the disease. HLA-B5 was confirmed as a marker of severe prognosis. Cardiolipin autoantibodies were associated with cutaneous vasculitis and superficial thrombophlebitis was correlated with systemic vessel involvement.
Adolescence ; Adult ; Aged ; Behcet's Syndrome/epidemiology* ; Behcet's Syndrome/complications ; Child ; Child, Preschool ; Europe/epidemiology ; Female ; Germany/epidemiology ; Human ; Male ; Middle Age ; Prognosis

The clinical features of cutaneous manifestations in 411 patients with Behcet's syndrome were studied. 302 patients (73.5%) had skin lesions. The frequency with which the following skin lesions were noted was, in decreasing order; erythema nodosum-like lesion, papulopustular eruption, erythema multiforme-like lesion, thrombophlebitis, ulcer and Sweet's syndrome-like lesion. More than two types of skin lesions were seen in 86 patients (28.5%), the combination of the skin lesions being in decreasing order, erythema nodosum-like lesion and papulopustular eruption; erythema multiforme-like lesion and papulopustular lesion; erythema nodosum-like lesion and thrombophlebitis. A skin pathergy test was performed on 245 patients, and a positive reaction was seen in 97 patients (39.6%). This study showed the high incidence, wide spectrum and importance of skin lesions as a major symptom in Behcet's syndrome.
Adolescent ; Adult ; Aged ; Behcet Syndrome/complications* ; Behcet Syndrome/epidemiology ; Child ; Female ; Human ; Male ; Middle Age ; Skin Diseases/classification ; Skin Diseases/epidemiology* ; Skin Diseases/etiology

Behcet's disease is a chronic relapsing multisystemic inflammatory disorder characterized by four major symptoms (oral aphthous ulcers, genital ulcers, skin lesions, and ocular lesions) and occasionally by five minor symptoms (arthritis, gastrointestinal ulcers, epididymitis, vascular lesions, and central nervous system symptoms). Although the etiology of Behcet's disease is still unknown, there have been recent advances in immunopathogenic studies, genome-wide association studies, animal models, diagnostic markers, and new biological agents. These advances have improved the clinical understanding of Behcet's disease and have enabled us to develop new treatment strategies for this intractable disease, which remains one of the leading causes of blindness.
Animals ; *Behcet Syndrome/epidemiology/immunology/therapy ; Biological Therapy/*trends ; Disease Models, Animal ; Humans ; Prevalence ; *World Health

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*

This study was undertaken to evaluate the clinical features of a cohort of Behcet's disease (BD) followed up from January 1997 to July 2001 in Yongdong districts in Korea, and to compare the results with the literature. Overall features of clinical manifestations were similar to those described in the literature. However, the frequency of gastrointestinal (GI) ulcerations was much higher than those of other Korean studies. The Korean studies including ours revealed a lower frequency of vascular lesions and epididymitis compared with studies of other countries. The most common site and pattern of inflammatory arthritis were knees and monoarticular involvement, respectively. In addition, in most patients, the ocular lesions involved the posterior uveal tract, and the terminal ileum and cecum were the most common sites of GI involvement. Patients with ocular lesions or GI lesions showed a good prognosis during the follow-up. The HLA-B51 antigen was positive in 50.7% of patients, and it was more commonly found in patients with a familial BD.
Adult ; Age Factors ; Arthritis/*epidemiology ; Behcet Syndrome/*epidemiology ; Cohort Studies ; Eye Diseases/epidemiology ; Female ; Follow-Up Studies ; Gastrointestinal Diseases/epidemiology ; Humans ; Korea ; Male ; Middle Aged ; Sex Factors

Esophageal involvement in Behcet's disease is very rare, and normally is observed as aphtosis and esophagitis, but serious complications such as erosions, perforations, and stenosis may occur. We carried out this prospective study to evaluate the prevalence of esophageal involvement in Behcet's disease and to establish if routine endoscopy and/or manometry are necessary. Twenty-three patients who fulfilled the diagnostic criteria of the international study group for Behcet's disease were enrolled. None were taking drugs or had disease that might produce esophageal abnormalities or alter any existing changes due to the Behcet's disease itself. Twenty- three patients underwent esophagogastroduodenoscopy by a single observer. Esophageal biopsies were performed in thirteen patients and esophageal manometry in twenty. At the beginning of the study, the disease activity was defined by the presence of more than one symptom related to Behcet's disease, and upon the classification of Behcet's disease. Of the twenty three patients enrolled two were excluded from final analysis because of the presence of hiatus hernia. Thus, 13 men and 8 women, ranging in age from 20 to 63 years with a mean age of 36.2 years were included. Ten patients (47.6%) had active disease and four (19%) complained of upper gastrointestinal symptoms at the time of the study. Fourteen patients had endoscopic, manometric and/or microscopic abnormalities. Esophageal manometry was performed in twenty patients and was abnormal in 7 cases (35%). Esophageal biopsies were done in 13 patients and revealed histopathological abnormalities in 5 cases. Microscopic findings showed vasculities in one case, and non-specific inflammatory infiltration mainly consisting of neutrophils in 4 cases. Our results suggest that the prevalence of esophageal involvement in Behcet's disease is rather high and occur even in asymptomatic patients, but that this usually does not result in specific abnormalities.
Adult ; Behcet Syndrome/*complications ; Esophageal Diseases/*epidemiology ; Esophagoscopy ; Esophagus/pathology ; Female ; Human ; Male ; Manometry ; Middle Age ; Prevalence ; Prospective Studies

PURPOSE: Behcet's disease (BD) is a chronic inflammatory disease characterized by orogenital ulcers, skin and ocular lesions, in addition to articular, vascular, and neurologic symptoms. Carpal tunnel syndrome (CTS), can also occur in BD patients secondary to inflammation in the connective tissues, vessels, and tendons, as well as nerve involvement in BD itself. However, reports of patients who have CTS in BD are rare. The aim of this study was to evaluate the clinical characteristics of CTS in BD patients. MATERIALS AND METHODS: Retrospective analysis of the medical records of 1750 BD patients, and 14 (0.8%) BD patients who were diagnosed with CTS was performed at the BD Specialty Clinic of Severance Hospital. Patient demographics, disease activity/severity for both diseases, and the clinical characteristics of CTS in BD were recorded and analyzed. RESULTS: All 14 BD patients with CTS were women. Twelve patients (85.7%) were diagnosed with active BD. The CTS was mild in 8 patients (57.2%), moderate in 3 patients (21.4%), and severe in 3 patients (21.4%). Ten patients (71.4%) had BD prior to the diagnosis of CTS, and these 10 patients all had active BD. CONCLUSION: CTS can occur as a result of the inflammation associated with BD and can also be the presenting symptom of nerve involvement in BD. CTS can also develop as the initial symptom of BD. Therefore, a higher degree of suspicion should be maintained for CTS in patients with BD and vice versa; however, the exact relationship is uncertain.
Adult ; Age Distribution ; Aged ; Behcet Syndrome/complications/diagnosis/*epidemiology ; Carpal Tunnel Syndrome/diagnosis/*epidemiology ; Female ; Humans ; Inflammation ; Male ; Middle Aged ; Retrospective Studies ; Sex Distribution