Epididymo-Orchitis in Bechet's Disease: A Review of the Wide Spectrum of the Disease.

 Behçet's disease is a chronic, recurrent, inflammatory disorder characterized by orogenital ulcers and skin lesions; serious manifestations also include ocular, large vessel, gastrointestinal and neurological involvement. Genetic and unknown environmental factors modify the wide clinical spectrum of the disease. During the long clinical course of the disease, testicular and epididymal involvement has been reported, with scrotal pain and swelling being the most common symptoms. In this review, we discuss the various aspects of epididymo-orchitis in Behcet's disease patients, and we evaluate the diagnostic approaches as well as the empirical therapeutic modalities of this entity.

Gastrointestinal manifestations of Behçet's disease: advances in evaluation and management.

Behçet's disease is a chronic, recurrent, inflammatory disorder characterized by orogenital ulcers and skin lesions; serious manifestations also include ocular, large vessel, gastrointestinal and neurological involvement. Genetic and unknown environmental factors customise the wide clinical expression of the disease. Gastrointestinal involvement is not unusual, albeit with a highly variable frequency among different ethnic populations. However, given the fact that gastrointestinal symptoms such as reflux, bleeding, diarrhoea are common in the general population, their clinical significance needs to be carefully interpreted. Apart from mouth the ileocecal area is typically involved, but inflammatory and/or vasculitic lesions may affect any part of the gastrointestinal tract. Complications such as perforation carry high morbidity rates and even mortality. Herein, we review all available information pertinent to gastrointestinal involvement of Behçet's disease and discuss the published advances in evaluation and its empirical management, including anti-TNF biologic therapies.

Etiopathogenesis of Behçet's disease with emphasis on the role of immunological aberrations.

Behçet's disease (BD) is a chronic multisystemic inflammatory disorder of unknown origin consisting of oral aphthous ulcers, ocular symptoms, skin lesions, and genital ulcerations. It has many features in common with systemic vasculitides and is more prevalent in countries along the ancient Silk route. Immune-mediated mechanisms play a major role in the pathogenesis of the disease, and inflammatory mediators are also involved. BD is not considered to be an autoimmune disorder, and the character of the disease needs to be clarified. Immunological aberrations in BD have been extensively studied by many investigators; genetic factors have been related to disease susceptibility, but their exact role in the development of disease is uncertain. Environmental factors such as infectious agents have also been implicated in the etiology of BD. However, the etiopathogenesis of the disease remains to be elucidated. Factors involved in the immunopathogenesis of BD with emphasis on the role of immunological aberrations are analyzed in this review.

Behçet's disease: recent advances in early diagnosis and effective treatment.

Behçet's disease (BD) is a chronic, multisystem, inflammatory disorder that is classified among the systemic vasculitidies. Its cause has yet to be determined, but genetic and environmental factors and immune dysregulation are thought to play a role in its pathogenesis. The diagnosis of the disease and recognition and separation from disorders that may mimic BD may be difficult, especially in areas where the disease is uncommon. Certainty as to the best treatments for the various manifestations of BD is often unclear due to limited randomized controlled trials. However, existing consensus statements and expert opinions should help to guide therapy. This review examines important recent contributions to help the clinician more readily recognize patients with the disease and institute appropriate care.

Aortic elastic properties and left ventricular diastolic function in patients with Adamantiades-Behcet's disease.

OBJECTIVES: We investigated whether Adamantiades-Behcet's disease (ABD) is related to impaired aortic (Ao) elastic properties and left ventricular (LV) function. BACKGROUND: Adamantiades-Behcet's disease is an inflammatory disorder characterized by vasculitis leading to vascular complications and, rarely, myocarditis. METHODS: We studied 82 patients with ABD (age: 40 +/- 12 years) and 24 normal control subjects by echocardiography. Abdominal Ao diameter (mm/m(2)) and Ao elastic indexes--namely, Ao strain (%), distensibility (cm(2) x dyn(-1)x 10(-6)), stiffness index, and pressure strain modulus (Ep) (cm(2) x dyn(-1) x 10(-6))--were calculated from the echocardiographically derived thoracic Ao diameters (mm/m(2)), and the measurement of pulse pressure obtained by cuff sphygmomanometry. Isovolumic relaxation time (IVRT) (ms), deceleration time (DT) (ms), and flow propagation velocity (FPV) (cm/s) were measured by Doppler echocardiography to assess diastolic LV function. The duration of disease and presence of vascular complications were noted. RESULTS: Patients versus control subjects had increased Ao diameters (p < 0.01), lower mean Ao strain and distensibility (4 vs. 9 and 1.4 vs. 3.4, respectively, p < 0.01), higher mean aortic stiffness index and Ep (15.6 vs. 6 and 1.17 vs. 0.44, respectively, p < 0.01), and impaired IVRT and FPV (p < 0.01). Aortic function indexes were related to the duration of disease (p < 0.01) and increased DT (p < 0.01). Deceleration time >190 ms predicted vascular complications with 80% sensitivity and 71% specificity (odds ratio 6.52 [confidence interval: 2.23 to 19.03]). CONCLUSION: Aortic elastic properties and diastolic LV function are impaired in patients with ABD and are interrelated. The link between diastolic LV dysfunction and vascular complications suggests the presence of a common pathophysiologic pathway and provides a possible marker of risk for vascular disease.