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.     

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.     

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.     

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.     

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.     

Epidemiology and clinical course of Behçet's disease in the Reggio Emilia area of Northern Italy: A seventeen‐year population‐based study

Objective

To investigate the epidemiology and clinical course of Behçet's disease (BD) over a 17‐year period in a defined area of northern Italy.

Methods

All patients with incident BD diagnosed over a 17‐year period (from January 1, 1988 to December 31, 2004) living in the Reggio Emilia area were identified through the following sources: physicians at Reggio Emilia Hospital, medical practitioners, and community‐based specialists. We identified all patients registered in a centralized index and in the Reggio Emilia district database for rare diseases. Patients were followed up from the time of diagnosis until either their death or April 1, 2005.

Results

Eighteen patients (9 men and 9 women) had complete BD. Mean ± SD age at diagnosis was 33 ± 7 years. The incidence rate of BD was 0.24 per 100,000. The prevalence of BD on January 1, 2005 was 3.8 per 100,000. No patients died during the followup period. Although all patients developed oral ulceration during the disease course, 22.2% had no oral lesions at disease onset. Eye disease occurred in 55.6%. Ocular disease was more common in men and appeared at disease onset or within the first few years of disease onset (median 3 years). Only 1 patient had loss of useful vision in at least 1 eye at the end of followup. In all affected patients, visual acuity improved once treatment was started.

Conclusion

This population‐based study is the first to report the prevalence and incidence of BD in Italy. In Italian patients, BD is nonfatal and the prognosis of eye disease is good.