Left ventricular intracardiac thrombus in a patient with Behçet disease successfully treated with immunosuppressive agents without anticoagulation: a case report and review of the literature.

Behçet disease (BD) is a chronic multisystem disorder with vasculitis underlying its systemic manifestations. Cardiac involvement and particularly left ventricular intracardiac thrombus are rarely diagnosed in the course of BD and are often associated with poor prognosis. The causes of intracardiac thrombi are unknown. It is plausible that specific proinflammatory pathways resulting in the endothelial cell injury and hypercoagulation contribute to the formation of thrombotic masses in the heart. Known thrombophilic factors such as methylenetetrahydrofolate reductase gene mutations, factor V Leiden mutation, proteins S and C, antithrombin III, activated protein C resistance, and antiphospholipid antibodies may contribute to the formation of intracardiac thrombi in BD. We report a case of a 24-year-old male patient with BD presented with left ventricular thrombus. Transthoracic echocardiography allowed to describe and monitor such a rare cardiac manifestation of the disease. A combination of high-dose corticosteroid and azathioprine successfully dissolved intracardiac thrombus within ten days without anticoagulation.

Behçet's disease and comorbidity: internal case reports and literature review.

The aim of this study was to determine the clinical characteristics of two patients with malignancy related to Behçet's disease. We reviewed literary-reported cases of Behçet's disease related to myelodysplastic syndrome, neoplasm and leukemia. Our own observation included two cases of comorbid conditions of Behçet's disease. The first patient with Behçet's disease had been examined concurrent development of intestinal tumor. The second one was diagnosed to have chromic myeloleukemia. The malignant transformation of Behçet's disease patients, both solid and hematological, is rather rare. Further accumulation of relevant date and its analysis will allow to find out the relationship between malignant disease and chemotherapy in Behçet's disease.

Single center study on ethnic and clinical features of Behcet's disease in Moscow, Russia.

For the purpose of investigating Behcet's disease (BD) in Russia, 250 consecutive patients (177 men and 73 women) diagnosed with BD between 1990 and 2010 at the Research Institute of Rheumatology, Russian Academy of Medical Sciences in Moscow were enrolled in this study. The ethnic backgrounds of the patients were reported as follows: 23.2% (58 cases) from Russia, 12.8% (32 cases) from Azerbaijan, 14.4% (36 cases) from Armenia, 8.8% (22 cases) from Chechnya, and 21.6% (55 cases) from Dagestan. The remaining 19.2% (48 cases) were from other regions or of unknown origin. More than half (57.6%) of the Behcet's disease patients originated from Central Asia, specifically Azerbaijan, Armenia, Chechnya, and Dagestan. The mean age at disease onset was 31.5 ± 9.38 (13-60) years old, and the most typical initial manifestations were oral aphthous ulcers. Patients aged 20-39 years old were more commonly affected and displayed a wide clinical spectrum of the disease, with varieties of severe internal organ involvement. The manifestations observed throughout the course of the disease included oral aphthous ulcers (100%), various cutaneous lesions (88.8%), genital ulcers (81.2%), and ocular lesions (54.0%). Besides these, many organs/systems were implicated in patient cases, namely joint (53.2%), vascular (25.2%), neurological (8.0%), gastrointestinal (25.2%), and cardiac (5.6%) systems. Involvements of ocular (p < 0.01) and skin (p < 0.01) lesions were more frequent in men than in women. HLA-B51 and HLA-A26 typing was performed in 127 patients and 508 healthy controls. HLA-B51 was found in 63.0% of BD patients compared to 20.7% of the healthy control subjects (p < 0.001), and HLA-A26 was present in 11.3% of BD patients and 18.9% of the control group. This study shows the presence of BD in Russia, and it is suggested that its prevalence in Central Asian people is much higher than that in White Russian.

Catalytic antibodies in clinical and experimental pathology: human and mouse models.

Most of the data accumulated through studies on natural catalytic autoantibodies indicate that production scales up markedly in pathological abnormalities. We have previously described an increased level of DNA-hydrolyzing autoantibodies in the sera of patients with various autoimmune disorders [systemic lupus erythematosus (SLE), rheumatoid arthritis, scleroderma], HIV infection and lymphoproliferative diseases accompanied by autoimmune manifestations. In the present study, we show that an increased level of catalytic activity of autoantibodies can be observed in the sera of autoimmune mice, thus providing a fundamental insight into the medical relevance of abzymes. Polyclonal autoantibodies purified from sera of NZB/W, MRL-lpr/lpr and SJL/J mice show proteolytic and DNA-hydrolyzing activities, as opposed to those harvested from non-autoimmune BALB/c mice. The expressiveness of the catalytic activity was strongly dependent on the age of the animal. The highest levels of catalytic activity were found in the sera of mice aged between 8 and 12 months; the lowest level was typical of younger animals whose age ranged from 6 to 8 weeks. Specific inhibition assays of the catalytic activities were performed to throw light on the nature of the abzyme activity. Within a cohort of aging animals, a strong correlation between marked autoimmune abnormalities and levels of catalytic activities has been established. Nonimmunized SJL/J mice revealed specific immune responses to myelin basic protein (MBP), skeletal muscle myosin (skMyo) and cardiac myosin (Myo), and highly purified antibodies from their serum show specific proteolytic attack against the target antigens. This finding prompted us to undertake a more detailed study of specific antibody-mediated proteolysis in diseased humans. A targeted catalytic response was originally demonstrated against MBP and Myo in multiple sclerosis and myocarditis patients, respectively.