Behcet’s disease: from east to west

Abstract

Behcet's disease (BD) is classified among vasculitides. There are actually five nationwide surveys of BD: Iran, Japan, China, Korea, and Germany. Among case series, four are on more than 200 cases (Turkey, Morocco, Tunisia, and UK). BD was classically seen around the Silk Route. Now, it is seen everywhere. The male to female ratios were, respectively (in nationwide surveys), 1.19, 0.98, 1.34, 0.63, and 1.40 to 1. The mean age at onset was 26.2, 35.7, 33.8, 29, and 26 years. Major manifestations were seen, respectively, in nationwide surveys: mucous membrane (oral aphthosis in 97%, 98%, 98%, 99%, and 98%; genital aphthosis in 65%, 73%, 76%, 83%, and 64%); skin manifestations (pseudo-folliculitis in 57%, N/A, 31%, N/A, and 62%; erythema nodosum in 22%, N/A, 38%, N/A, and 42%; ocular manifestations in 55%, 69%, 35%, 51%, and 53%). Minor manifestations were seen, respectively, in nationwide surveys: joint manifestations in 33%, 57%, 30%, 38%, and 53%; neurological manifestations in 9%, 11%, 6.5%, 4.6%, and 11%; gastrointestinal manifestations in 7%, 15.5%, 9%, 7.3%, and 12%; vascular involvement in 8.9%, 8.9%, 7.7%, 1.8%, and 13%; pulmonary manifestations in 0.3%, N/A, 2.2%, N/A, and 3.6%; cardiac manifestations in 0.5%, N/A, 4%, N/A, and 3.2%. Laboratory tests are not useful except the pathergy test, which was positive in 54%, 44%, N/A, 40%, and 34% of cases. ESR was normal in many patients. Diagnosis is based upon clinical manifestations. The International Criteria for Behcet's Disease (ICBD, 2006) may be of help, having a sensitivity of 98.2% and a specificity of 95.6% in Iranian patients.

Gene expression profiling and association studies implicate the neuregulin signaling pathway in Behçet's disease susceptibility

“…The distribution of clinical symptoms in the Iranian BD patients (e.g., 98.7 % with oral aphthosis, 62.3 % with genital aphthosis, 55.2 % with skin lesions, 59. 7 % with ocular lesions) is in line with what has been observed for larger datasets [16], suggesting that this is a representative group. Cases and controls were matched for age-at-examination (mean AAE±SD of 39.1±11.0 years in BD and 40.0±12.3 years in controls, P=1.93E-01).…”

Behçet's disease: Successful treatment with infliximab in 7 patients with severe vascular manifestations. A retrospective analysis

“…Disease classification is based on the most prevalent disease manifestations (7). Vasculitis with a prevalence between 1.8% and 13% is not included (8). Severe forms of vasculitis are characteristic for young male individuals, leading to an increased mortality, especially in cases of arterial involvement as is markedly represented by our male patients (9).…”

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

“…The prevalence in Turkey ranges from 200 to 4,210 per million, whereas the prevalence in other European countries is only 24 per million [165]. Onset is generally in the third to fifth decades of life; men are affected more often than women and tend to have a worse outcome [166,167]. The vascular inflammation in Behçet's primarily affects the venous system.…”

“…Neurological complications of Behçet's occur in approximately 10% of patients, which are more commonly seen in men and tend to occur after several years of having the disease [166,167]. Most patients have parenchymal involvement of the brain due to inflammation of the small veins [166].…”