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.

Diffuse Calcifications of the Spleen in a Woman with Systemic Lupus Erythematosus.

Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disease, which affects a wide variety of organs including the spleen. Splenic involvement in SLE includes conditions such as splenomegaly, hyposplenism, infarction, and spontaneous rupture. However, only a few cases of splenic calcifications in patients with SLE have been reported. Herein, we present a case of a 24-year-old female diagnosed with SLE, in which we found diffuse splenic calcifications. The unique pattern of splenic calcifications in SLE contributes to the differential diagnosis from other conditions such as infections and other connective tissue diseases, which also cause calcifications in the spleen.

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.

A single infliximab infusion vs corticosteroids for acute panuveitis attacks in Behçet's disease: a comparative 4-week study.

OBJECTIVE: To compare a single infusion of the anti-TNF antibody infliximab vs CSs for acute panuveitis attacks in Behçet's disease (BD). METHODS: A prospective, observational study of patients with panuveitis, who received either an infliximab infusion (5 mg/kg, 19 eyes) or high-dose methylprednisolone intravenously (1 g/day for 3 days, 8 eyes), or intra-vitreal triamcinolone acetonide (4 mg, 8 eyes) at attack's onset. Baseline maintenance therapy remained unchanged during the following 30 days. Visual acuity, anterior chamber cells, vitreous cells and inflammation of the posterior eye segment were assessed at baseline and at Days 1, 7, 14 and 29 (±1) post-treatment. RESULTS: While no significant differences were noted between i.v. and intra-vitreal CSs, infliximab was faster than CSs in decreasing total ocular inflammation scores and fundus inflammation scores (P = 0.01 and P < 0.0001 for treatment × time(2) interaction, respectively, using generalized estimating equation analysis). Independently of time, infliximab was superior to CSs in clearing retinal vasculitis (P < 0.003), as well as in resolution of retinitis (P = 0.008) and cystoid macular oedema (P < 0.007). Moreover, a faster regression of cystoid macular oedema was observed with infliximab compared with CSs (P < 0.03). The beneficial effects of the three treatment modalities on visual acuity were comparable from baseline to the end of follow-up. No side effects were noted with infliximab or methylprednisolone, whereas intra-vitreal triamcinolone acetonide caused ocular hypertension in four of the eight eyes, requiring surgical intervention in two. CONCLUSION: A single infusion of infliximab should always be considered, even as an adjunct therapy, for the control of acute panuveitis attacks in BD.

Genome-wide association study identifies variants in the MHC class I, IL10, and IL23R-IL12RB2 regions associated with Behçet's disease.

Behçet's disease is a genetically complex disease of unknown etiology characterized by recurrent inflammatory attacks affecting the orogenital mucosa, eyes and skin. We performed a genome-wide association study with 311,459 SNPs in 1,215 individuals with Behçet's disease (cases) and 1,278 healthy controls from Turkey. We confirmed the known association of Behçet's disease with HLA-B*51 and identified a second, independent association within the MHC Class I region. We also identified an association at IL10 (rs1518111, P = 1.88 x 10(-8)). Using a meta-analysis with an additional five cohorts from Turkey, the Middle East, Europe and Asia, comprising a total of 2,430 cases and 2,660 controls, we identified associations at IL10 (rs1518111, P = 3.54 x 10(-18), odds ratio = 1.45, 95% CI 1.34-1.58) and the IL23R-IL12RB2 locus (rs924080, P = 6.69 x 10(-9), OR = 1.28, 95% CI 1.18-1.39). The disease-associated IL10 variant (the rs1518111 A allele) was associated with diminished mRNA expression and low protein production.

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.

Arterial wave reflections are associated with left ventricular diastolic dysfunction in Adamantiades-Behçet's disease.

BACKGROUND: Adamantiades-Behçet's disease (ABD) is characterized by systemic vasculitis. We investigated whether aortic distensibility and arterial wave reflections are linked to left ventricular (LV) diastolic dysfunction in ABD. METHODS AND RESULTS: Eighty-two patients and 40 controls with similar atherosclerotic risk factors were examined by applanation tonometry of the radial artery (SphygmoCor) and echocardiography. Augmentation index (AI%) and arrival time (Deltat, ms) of reflected arterial waves as well as aortic distensibility (AoD) assessed by echocardiography were estimated. Doppler diastolic abnormalities were defined as proposed by the European Study Group on Diastolic Heart Failure by measurement of E/A ratio, isovolumic relaxation time, deceleration time, and flow propagation velocity. Patients had impaired central augmentation index (CAI), Deltat, and AoD compared with controls (P > .05). After adjusting for age, atherosclerotic risk factors, left ventricular mass, and medication the odds-ratio of AoD and CAI for left ventricular diastolic dysfunction was 0.664 (95%CI 0.449-0.982), P = .04, and 1.073 (95% CI 1.014-1.140), P = .001, respectively. The addition of CAI to the multivariable model including AoD significantly increased the power of the model for prediction of left ventricular diastolic dysfunction (-2 Log likelihood change = 18.8, P for change > .01). CONCLUSION: Augmentation index has a complementary value to aortic distensibility in the assessment of left ventricular diastolic dysfunction in ABD.

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.

Multiple myeloma associated with systemic lupus erythematosus.

A case of systemic lupus erythematosus (SLE) complicated by multiple myeloma is presented; the lupus diagnosis was put together with the diagnosis of myeloma but the symptoms had commenced a few years before. The putative mechanisms underlying this unusual combination are discussed.

Multiple myeloma associated with systemic lupus erythematosus.

A case of systemic lupus erythematosus (SLE) complicated by multiple myeloma is presented. The lupus diagnosis was established together with the diagnosis of myeloma but the symptoms had commenced a few years before. The putative mechanisms underlying this unusual combination are discussed.