Use of infliximab and other biologics in Behçet disease

Behçet disease is a multisystem vasculitis characterised by recurrent oral ulceration in conjunction with other manifestations. Neurological involvement or neuro‐Behçet disease is not common, but typically affects young men at its onset between the ages of 20 and 40 with significant long‐term morbidity and mortality. There is substantial case literature to support the use of tumour necrosis factor antagonists, notably infliximab, in the treatment of neuro‐Behçet disease.     

Intracardiac Mass as Initial Cardiac Manifestation of Behçet's Disease: Think before You Cut

Behçet's disease (BD) is a chronic multisystemic inflammatory disorder. Cardiac abnormalities including intracardiac thrombi have been described in up to 16% of cases. The clinical presentation of cardiac complications in BD may include fever, dyspnea, chest pain, hemoptysis, and edema. We present 2 cases of patients who underwent surgical excision of intracardiac masses thought to be intracardiac malignancies. Further pathological and clinical evaluation established intracardiac inflammatory masses due to BD as the final diagnosis. As intracardiac masses may be the presenting manifestation of BD, it is crucial for echocardiographers to consider BD in the differential diagnosis. A careful history and physical exam looking for signs and symptoms of BD is critical before considering surgical excision of unexplained intracardiac masses. If the final diagnosis is BD anti‐inflammatory therapy should be considered the basis of treatment.     

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.     

Successful use of infliximab in a patient with neuro‐Behçet’s disease

Behçet′s disease (BD) is a systemic vasculitic disorder of unknown aetiology characterised by recurrent oral and often genital ulcers, which may be associated with ocular, cutaneous, articular, neurological or vascular involvement. We report a 52‐year‐old woman diagnosed of neuro‐BD who was treated with infliximab with a dramatic response to this treatment.     

BENEFICIAL EFFECT OF ORAL MESALAZINE POWDER ADMINISTRATION ON GASTRIC INVOLVEMENT OF BEHÇET's DISEASE

Behçet's disease (BD) is a multisystem immune‐mediated inflammatory disorder that involves the gastrointestinal tract. Although intestinal BD chiefly affects the ileocecum and colon, it may involve the entire alimentary tract. Upper gastrointestinal involvements of BD are not common, and its treatment has been rarely reported. Mesalazine is known to be an effective anti‐inflammatory agent for treatment of distal intestinal lesions of inflammatory bowel disease by its delayed drug release system. Here we show a case of chronic active, steroid‐dependent BD involving the upper gastrointestinal tract who was treated with ground mesalazine powder. Endoscopy performed 8 weeks after the treatment showed marked improvement of the gastric ulcers. This is the first report of intestinal BD treated with oral administration of mesalazine powder, and our results suggest that it may be a beneficial and effective new therapy for the upper gastrointestinal lesion of BD.     

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.     

Serum homocystein level and vascular thrombosis in patients with Behçet disease

Objective: Behçet disease (BD) is a multisystemic inflammatory disorder of unknown aetiology that in some patients will present with thrombosis. However, the mechanism of thrombosis is unknown. In this study, we investigate the correlation between homocystein level in BD patients with vascular thrombosis. Patient and methods: One hundred and twenty‐three patients who fullfilled the criteria of the International Study Group for BD (n = 77, 62.6% females and n = 46, 37.4% males) were included in this cross‐sectional study. Patients were divided into two groups: with thrombosis (n = 23, 18.7%) and without thrombosis (n = 100, 81.3%). All patients underwent colour Doppler sonography for documentation of vascular thrombosis. Serum homocystein was measured by ELISA method, folate and B12 were measured by radioimmunoassay. Results: Patients with vascular involvement, vein and/or arterial thrombosis were enrolled. Mean serum homocystein level was significantly higher in the group with thrombosis (P = 0.012) and in male patients than females (18.10 ± 5.5 vs. 13.21 ± 4.1). In multiple regression analysis, the only factors that persisted for thrombosis were age and sex (P = 0.02, P < 0.0001, respectively). Serum folate and B12 were not correlated with thrombosis in BD. Conclusions: In this study, we found that mean homocystein levels were higher in BD patients with thrombosis and it may have a role in the process of thrombosis in BD.     

Clinicopathologic features and outcomes of neuro-Behçet disease in Spain: a study of 20 patients

BackgroundTo describe the clinical characteristics and evolution of a series of adult patients hospitalized for neuro-Behçet disease (NBD).MethodsConsecutive patients admitted for NBD in a teaching hospital were retrospectively selected. Disability at discharge and during follow-up was graded with the modified Rankin Scale, and outcome classified as good or poor (grades 3–6).ResultsTwenty patients were included (M/F, 13/7). Mean age at NBD diagnosis was 36.3 years. Nineteen patients had other manifestations of Behçet disease (BD) before NBD developed, but only 7 met the complete diagnostic criteria for BD. Fever, headache, motor weakness, and cranial nerve palsy were each present in approximately 60% of patients. There was a low prevalence of behavioral changes (5%), seizures (5%), and sphincter incontinence (0%), and a relatively high prevalence of meningism (25%). Non-neurologic manifestations of BD were concurrently detected in 15 patients (75%). 80% had parenchymal involvement. Brain biopsies during 5 attacks showed perivascular lymphocytic infiltration with reactive astrocytosis, but no frank vasculitis. During a mean follow-up of 6.3 years per patient, 12 had at least one relapse. In total, there were 22 relapses; all but two were in the same location and were symptomatically similar in each patient. At the end of follow-up, 7 patients (35%) had a poor outcome, including 4 who died.ConclusionRecording of previous manifestations of BD and a physical examination to detect concomitant systemic manifestations of BD may help establish an early diagnosis of NBD. Relapses frequently occurred in the same location. No frank vasculitis was present in brain biopsies.