Behçet's Disease

Kaklamani, VG; Vaiopoulos, George; Kaklamanis, Ph. G.

doi:10.1016/s0049-0172(98)80001-2

Cited by 396 publications

(251 citation statements)

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“…In a hospital-based series, KuralSeyahi et al (7) observed an increased mortality in patients with BD, in particular among young males, which tended to decrease with the passage of time. However, in agreement with our data, 2 other studies reported no increase in mortality in patients with BD (42,43). Turning to morbidity, we looked specifically at inflammatory ocular disease, because this is both a frequent and a potentially serious complication of BD with a significant risk of visual loss (1,2).…”

Section: Seventeen-year Study Of Behçet's Disease In Italysupporting

confidence: 89%

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

Salvarani¹,

Pipitone²,

Catanoso³

et al. 2007

Arthritis & Rheumatism

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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.

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Section: Seventeen-year Study Of Behçet's Disease In Italysupporting

confidence: 89%

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

Salvarani¹,

Pipitone²,

Catanoso³

et al. 2007

Arthritis & Rheumatism

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“…Its clinical expression may be dominated by orogenital ulcerations, skin lesions, intraocular inflammation and less commonly by arthritic, vascular, gastrointestinal, and neurologic manifestations [1][2][3]. The complete etiology and pathogenesis of BD remain unknown, and the clinical course is characterized by exacerbations and remissions of unpredictable duration and frequency.…”

Section: Introductionmentioning

confidence: 99%

Anti-Alpha-Enolase Antibody as a Serologic Marker and Its Correlation with Disease Severity in Intestinal Behçet’s Disease

Shin

Kim

et al. 2010

Dig Dis Sci

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Background Intestinal Behçet's disease (BD) is a chronic inflammatory bowel disease, as are Crohn's disease (CD) and ulcerative colitis (UC). But unlike CD and UC, serologic markers for intestinal BD are not well known. Recently, antia-enolase antibody (AAEA) has been detected in sera from BD patients. Aims The aim of this study was to evaluate the prevalence of AAEA in intestinal BD and its clinical correlations. Methods The study sample included 80 patients with intestinal BD and 23 healthy controls. IgM AAEA was detected by ELISA. The positivity of IgM AAEA was defined as an optical density greater than three standard deviations above the mean of the control sera. Other parameters, such as demographic information, subtype of BD, colonoscopic findings, disease severity and treatment modality, were analyzed retrospectively. Results The prevalence of IgM AAEA was 67.5% in intestinal BD and 0% in the control group. The positivity rate of IgM AAEA was higher in complete or incomplete BD than in suspected BD (77.5% vs. 51.6%, P = 0.016). The mean HBI score was higher in antibody positive patients than in antibody negative patients (5.60 vs. 4.61, P = 0.003). The cumulative probability of steroid use for aggravation of intestinal and extra-intestinal symptoms was higher in antibody positive patients than in antibody negative patients (P = 0.012). The number of patients with systemic involvement was higher in the AAEA positive group than in the negative group. Conclusions Monitoring IgM AAEA may be helpful for diagnosis of intestinal BD and could be used to predict clinical course and disease severity.

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“…Behçet's disease (BD) is a chronic inXammatory disorder aVecting various organ systems, characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions [1,2]. All these common manifestations are self-limiting except for the ocular attacks.…”

Section: Introductionmentioning

confidence: 99%

“…All these common manifestations are self-limiting except for the ocular attacks. Repeated attacks of uveitis can cause blindness [1][2][3]. Involvement of the gastrointestinal tract, central nervous system, and large vessels is less frequent, although it can be life threatening [1,2,[4][5][6].…”

Section: Introductionmentioning

confidence: 99%

One year study of efficacy and safety of infliximab in the treatment of patients with ocular and neurological Behçet’s disease refractory to standard immunosuppressive drugs

Giardina

Ferrante²,

Ciccia³

et al. 2009

Rheumatol Int

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The aim of the study was to assess the long-term eYcacy and safety of InXiximab therapy in the treatment of patients with Behçet's disease refractory to standard immunosuppressive agents. Twenty-one patients that did not respond to corticosteroids and to at least one immunosuppressant (cyclosporin, methotrexate, azathioprine, cyclophosphamide) for the presence of ocular and/or CNS involvement were enrolled. Eighteen patients completed the study up to 54 weeks. Stable doses of prednisone (<10 mg/day) were permitted, immunosuppressants were discontinued at least 4 weeks prior baseline visit. The patients received three infusions of 5 mg/kg InXiximab (at weeks 0, 2 and 6) and then infusions of 5 mg/kg InXiximab every 8 weeks. At each visit data on clinical symptoms, response to therapy and adverse events were collected. The primary outcome of interest was to assess the clinical eYcacy (total or partial recovery) of inXiximab. Secondary end points were to evaluate quality of life and to monitor the safety of the drug. Eighteen patients achieved a total remission. Two patients achieved a partial remission and relapsed after 3 months from discontinuation of therapy. InXiximab was well tolerated throughout the study. A case of non-Hodgkin lymphoma was observed within 6 months.Minor side eVects were headache, dizziness, tachycardia that regressed spontaneously and did not entail interruption. Anti-nuclear antibodies were not detected during the period of observation.

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Behçet's Disease

Abstract: BD is a widespread vasculitis affecting young people and involving concurrently or consecutively nearly all organs and systems. Treatment results in better prognosis even when vital organs are involved.

Cited by 396 publications

References 0 publications

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

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

Anti-Alpha-Enolase Antibody as a Serologic Marker and Its Correlation with Disease Severity in Intestinal Behçet’s Disease

One year study of efficacy and safety of infliximab in the treatment of patients with ocular and neurological Behçet’s disease refractory to standard immunosuppressive drugs

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