Síndrome de Behcet / Síndrome de Behcet

El síndrome de Behcet, también conocido como enfermedad de Behcet es un proceso autoinflamatorio crónico de baja frecuencia y etiología desconocida. Es una vasculitis que afecta arterias y venas de todos los calibres, provoca una alteración de la función endotelial y se expresa clínicamente con lesiones orgánicas en varios niveles. Tiene una mayor incidencia en los países localizados en la antigua ruta de la seda (mediterraneo del este, oriente medio y este de Asia). En su fisiopatogenia intervienen factores genéticos, microbianos e inmunológicos. Los síntomas varían de persona a persona y de acuerdo a las regiones geográficas, pero los más comunes son las úlceras orales y genitales, inflamaciones oculares (uveítis, retinitis e iritis), lesiones de piel y artritis. En algunos casos pueden presentarse alteraciones del sistema nervioso central, del tracto digestivo y renal. Como guía para el diagnóstico se emplean los criterios del grupo internacional de estudio de la enfermedad de Behcet. El tratamiento incluye medicamentos de uso tópico y sistémico, entre estos los más empleados son los esteroides, los inhibidores del factor de necrosis tumoral y los inmunosupresores(AU)

Behcet's syndrome, also known as Behcet's disease is a chronic autoinflammatory process of low frequency and unknown etiology. It is a vasculitis affecting arteries and veins of all sizes that causes an alteration of endothelial function and is expressed clinically by organ damage at various levels. It has a higher incidence in countries located in the ancient Silk Road (East Mediterranean, Middle East and East Asia). Its pathogenesis involves genetic, microbial and immunological factors.Symptoms vary from person to person and according to geographical regions, but the most common are oral and genital ulcers, eye inflammation (uveitis, iritis and retinitis), skin lesions and arthritis. In some cases they can present central nervous system, gastrointestinal tract and kidney. As a guide to the diagnosis criteria of International Study Group of Behcet's disease are used. Treatment includes topical and systemic drugs. The most commonly used are steroids, tumor necrosis factor inhibitors and immunosuppressants(AU)

Behcet's disease in India: A dermatological perspective.

Background : Behcet's disease (BD) is a chronic, recurrent, multi-system inflammatory disorder involving mucocutaneous (MC), ocular, intestinal, articular, vascular, urogenital and neurologic systems. BD occurs with a high prevalence in the Mediterranean population. There is scarcity of clinical data on BD from India with only three case series in the last two decades. Aims: To study demographic profile, clinical manifestations and treatment outcome of patients with BD presenting to the dermatologic clinic in a tertiary hospital in north India. Methods: Prospective analysis of all patients diagnosed to have BD between 1997 to 2011. Result: Twenty nine patients were diagnosed to have BD. The disease had a female preponderance (M:F = 1:3.8) with a mean age of disease onset of 27.4 (range 16-61) years. The prevalence of various MC and systemic manifestations are as follows: oral aphthae (100%), genital aphthae (93.1%), erythema nodosum (62%), papulopustular and acneiform lesions (31%), articular involvement (68.9%), ocular involvement (31%) and gastrointestinal (GI) involvement (3.4%) . Pathergy test positivity was observed in 31%. The treatment comprised of colchicine (16/29 patients), dapsone (7/29), dapsone with pentoxiphylline (3/29), systemic steroid (2/29), systemic steroid with methotrexate (1/29). Colchicine was effective and well tolerated in all patients. Conclusion: The disease occurs in a much milder form in India and is primarily mucocutaneous and arthritic. A high index of suspicion in patients with MC lesions may result in early diagnosis, management and prevention of complications of BD. We suggest colchicine as an effective and safe therapeutic option for MC and joint involvement.

Ocular manifestations of behcet's disease in Jordanian patients

To study the prevalence, manifestations and severity of ocular involvement of Behcet's disease in Jordanian patients. The study population consisted of 43 patients diagnosed to have Behcet's disease through Rheumatologist's examinations conducted at Jordan University Hospital between January 2002 and July 2009. The sample involved patients who displayed ocular manifestations. This included 18 patients; 12 males and 6 females with a mean age of 35 years [SD = 17.26]. Ophthalmological examinations and retrospective analysis of medical files were carried on. Ocular manifestations were seen in 41.9% of patients. The most common manifestation for Behcet's disease was vitritis with a prevalence of 55.6%, followed by anterior uveitis and retinal vasculitis [50% for each]. On the other hand, the most frequent complications involved were cataract, cystoid macular edema [CMO], posterior synechiae and glaucoma with a prevalence of [44.4%], [33.3%], [11.1%] and [5.6%], respectively. The prevalence and severity of ocular lesions in Behcet's disease is relatively low in Jordanian patients. This result indicates that early diagnoses and intervention might delay or even prevent vision loss for those patients.

New Insights in the Clinical Understanding of Behcet's Disease

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.

Neurological manifestations of behcet's disease

To determine the prevalence, clinical manifestations, and laboratory features of Neuro-Behcet's disease. This prospective study was carried out in the Behcet's Research Clinic in Shiraz [south-west Iran] and included the patients referred from 1990-1999. The patients' clinical records, images, CSF analyses, and electrodiagnostic studies were reviewed. Eighteen [15 males and 3 females] out of 690 Behcet's patients [2.6%, 95% CI = 1.4-3.8%] were found to have neurological involvement. The mean +/- standard deviation age of these patients was 34.7 +/- 8.6 years. All fulfilled the criteria of the International Study Group of Behcet's Disease. Central nervous system involvement was more common than peripheral nervous system manifestations. Headache, weakness, tingling, and numbness were the most common symptoms. Hyperreflexia, upward plantar reflex, and somatosensory findings were the most frequent signs. Hemispheral and brainstem stroke-like syndromes and cerebral venous thrombosis were the major neurologic presentations. There were also cases of myelitic, pure meningoencephalitic, amyotrophic lateral sclerosis-like, multiple sclerosis-like, and Guillain Barre syndromes. Neuro-Behcet's disease must be considered in the differential diagnosis of stroke in young adults, chronic meningitis, intracranial hypertension, multiple sclerosis, myelopathies, and peripheral neuropathies

Research report: Frequencies of mica gene polymorphism: a comparison between Indonesians on Bacan Island and suburban Japanese.

MHC class I chain related gene A (MICA) is located near the HLA-B gene on the short arm of human chromosome 6. In the transmembrane (TM) of region of MICA, there is a trinucleotide repeat (GCT/AGC) microsatellite polymorphism in exon 5. Five alleles with 4, 5, 6 and 9 repetitions or 5 repetitions with 1 additional nucleotide insertion (GGCT) are identified and they were named A4, A5, A5.1, A6, and A9 respectively. We report the allele frequencies of 127 Indonesians on Bacan Island and 250 Japanese in the Kanto area. From the genotyping result, the frequency among Indonesians was as follows: A4 15.4%, A5 26.0%, A5.1 16.5%, A6 5.5%, and A9 36.6%. The frequency among Japanese was as follows: A4 20.6%, A5 28.1%, A5.1 10.8%, A6 27.2%, and A9 13.2%. Allele 9 is significantly increased and allele 6 significantly decreased in Indonesians compared with Japanese subjects. The results suggested that MICA microsatellite polymorphism are quite different in each race. Among Indonesians, the frequency of MICA-A9 allele, which was reported to be negatively associated with Behçet's disease, was significantly higher, whereas the MICA-A6 allele frequency, which was reported to be positively associated with Behçet's disease, was significantly lower among Japanese.

Influence of Sex on Patients with Behcet's Disease in Korea

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.

Survey and Validation of the Criteria for Behcet's Disease Recently Used in Korea: a Suggestion for Modification of the International Study Group Criteria

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.

The Clinical Features of Behcet's Disease in Yongdong Districts: Analysis of a Cohort Followed from 1997 to 2001

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.

Epidemiological features of Adamantiades-Behcet's disease in Germany and in Europe

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.

Epidemiological and clinical features of Behcet's disease in Korea

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.

Behcet's disease in Oman

This is a study of 25 patients of Behcet's Disease [BD] who were followed from 1990 to 1996 at the dermatology department of Baushar and AI Nahdha hospital, Muscat. The diagnosis of the BD was based on the clinical criteria set by the International Study Group for Behcet's disease. Their presenting features, progress, complications as observed in Oman and the response to the various therapies are discussed. Ocular involvement has been the dreaded complication that has maximally debilitated the patients [13/25], i.e. 52% developed eye problems out of which 8 patients, i.e. 32%, eventually became economical blind. Chronic mouth ulceration was seen in 100%, while genital ulcerations were seen in 64% of the cases. The CNS was affected in 16%.Simuzi's classification of complete and partial Behcet's disease is highlighted for its significance of suspecting the cases earlier and in view of using immunosuppressive prophylaxis therapy that is supposed to prevent eye complications. The aim of this paper is to highlight the prevalence of this disease in this region and its high association of eye complications so that awareness is created which will lead to early suspicion and detection of such cases

Doença de Behçet: revisäo baseada na 6a. Conferência Internacional - Paris, 1993 / Behçet's disease: review established on 6th International Conference - Pris, 1993

Breve revisäo sobre aspectos etiopatogênicos, epidemiológicos, dermatológicos, critérios de classificaçäo e terapêutica da doença de Behçet, abordados na 6a. Conferência Internacional da Doença de Behçet, em Paris, França, em 30 de junho e 01 de julho de 1993

Cutaneous Manifestations of Behcet's Syndrome

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.