Behcet’s disease: global perspective

Abstract: Behcet’s disease (BD) is a vasculitis progressing by attacks and remissions. There are actually four nationwide surveys of BD; Iran (5059 patients), Japan (3316 patients), China (1996 patients), and Germany (590 patients). There are four major case series in the world, Turkey (2147 patients), Korea (1155 patients), Morocco (1034 patients), and UK (419 patients). Other series are based on less than 200 patients. Mucous membrane manifestations: Oral aphthosis is seen in 98% of patients (average from the above-mentioned countries). Genital aphthosis occurs less frequently (73%). Skin manifestations are seen in 74% of patients. Behcet’s pustulosis (pseudofolliculitis) is seen in 61% of patients in Iran. Erythema nodosum is seen in 22% of patients in Iran. They are more frequent in China and Korea. Ocular manifestations are seen in 51% of patients (anterior uveitis, posterior uveitis retinal vasculitis). Joint manifestations are seen in 39% of patients (arthralgia, monoarthritis, oligo/polyarthritis, ankylosing spondylitis). Neurological manifestations are seen in 7.3% of patients.They are mainly central manifestations due to parenchymal lesions. Gastrointestinal (GI) manifestations are seen in 9% of patients (produced by aphthous ulcers of the intestinal tract). Vascular involvement occurs in 11% of patients. Arterial involvement is rare. In Iran it is seen in 0.6% of patients. Deep vein thrombosis is seen in 6%, large vein thrombosis in 1.2%, and superficial phlebitis in 2.3%. Other lesions: Orchitis and epididymitis were seen in 5.6% of patients in Iran. Cardio-pulmonary manifestations were rare, seen in only 1.4% of patients in Iran. Laboratory findings: Erythrocyte sedimentation rate was normal in 43.5% of cases in Iran. Urinary abnormalities were infrequent and transient (9.8% in Iran). Proteinuria was seen in 2%, hematuria in 4.6%, leukocyturia in 5.1%, and cast in 0.3% of cases. Pathergy test was positive in 57.4% and HLA-B51 in 34% of patients. Conclusion: BD is a systemic disease with various manifestations. It progresses by attacks and remissions. Lesions usually heal without sequela, except for eyes, brain, and vascular system. The main manifestations are mucocutaneous. The major cause of morbidity is the ocular lesion, which leads to severe loss of vision or blindness. Keywords: Behcet’s disease, clinical manifestations, diagnosis, epidemiology, treatment.