Management of vascular Behçet's disease

Behçet's disease (BD) is a chronic, multisystemic, inflammatory disease characterized by recurrent attacks of mucocutaneous, ocular, musculoskeletal, vascular, central nervous system and gastrointestinal manifestations. Treatment of BD changes according to organ involvement, gender and age of the patient with no golden standard therapeutic regimen. Vascular involvement is observed in up to 40% of the patients with BD, especially in young males and is one of the major causes of mortality and morbidity. Glucocorticoids, azathioprine and cyclophosphamide are still recommended as the first‐line treatments in vascular BD. However, increasing data with the tumor necrosis factor inhibitors suggest that these agents may also be acceptable options for the management of refractory vascular BD in daily practice. Anticoagulant usage for vascular BD is also still controversial with limited data coming from retrospective studies. There is a clear need for randomized, controlled studies for the management of VBD.     

Colitis of Behçet's Syndrome

ABSTRACT A 25-year-old previously healthy man developed complete Behçet's syndrome during five weeks. His main complaint was diarrhea with blood. Ulcers resembling aphthous ulcers of the mouth were revealed in the transverse, left and sigmoid colon by X-ray and sigmoidoscopy. Prednisone therapy was initiated and resulted in complete clinical restitution within three weeks.     

HLA Antigens in a Family with Behçet's Syndrome

ABSTRACT A family is described in which 3 of 12 members suffered from Behçet's syndrome. Four members, including the three patients with Behçet's syndrome suffered from recurrent aphthous stomatitis. These four family members possessed the HLA-haplotype HLA A2, B15, Cw3, DR4. However, one relative had inherited the same HLA-haplotype without showing any symptoms of the disease. Genetic influence from the actual HLA-haplotype may interact in manifestation of the syndrome.     

Behçet's Disease and Close Contact with Pigs

ABSTRACT Six male patients out of seven with Behçet's disease were found to have a very similar and close contact with pigs and pork. These six cases are reported and we ask the question whether the contacts with pigs are just coincidental findings or whether an external agent, e.g. a virus, could be forwarded to man from pigs and pork. Immune complex associated vasculitis is described e.g. in patients with hepatitis B and the same mechanisms might be operating in patients with Behçet's disease.