Diagnosis of Behçet's disease: clinical characteristics, diagnostic criteria, and differential diagnoses.

Background: The diagnosis of Behçet disease (BD) is challenging in many cases. The purpose of this study was to describe the clinical characteristics of patients at a referral BD clinic.

Methods: In a retrospective study, we collected data from patients at a national referral Behçet clinic from November 2018-August 2019.

Behcet's disease in Iran: Analysis of 7641 cases.

To analyze Behcet's Disease (BD) in Iran, from 1975 to 2018, and compare to 35 large/small reports from other countries. Patients from all over Iran, when suspected, were sent to the BD Unit. The diagnosis was done by expert opinion.

Paediatric Behçet's disease in Iran: report of 204 cases.

Objectives: This study proposed to report the characteristics of paediatric Behçet's disease (PED-BD) in a cohort of patients from Iran's registry and compare them with different reports throughout the world.

Methods: From a cohort of 7504 Iranian patients with Behçet's disease those diagnosed before the age of 16 years were included in this study. Data were collected on a standard protocol comprising 105 items, including demographic features, type of presentation, and different clinical and laboratory findings.

High-dose intravenous steroid pulse therapy in ocular involvement of Behcet's disease: a pilot double-blind controlled study.

Aim: To evaluate the efficacy of intravenous high-dose pulses of methylprednisolone (IVPM) for treatment of ocular involvement in Behcet's disease (BD).

Method: In a double-blind control study, we randomized BD patients with posterior uveitis (PU) and/or retinal vasculitis (RV) into two groups. They received either IVPM (1000 mg methylprednisolone) or placebo for 3 consecutive days.

Behcet's disease: epidemiology, clinical manifestations, and diagnosis.

Behcet's Disease (BD) is classified among vasculitides. The aim of this review was to put together different known reports in order to help the reader to better understand the disease, to avoid the frequent misdiagnosis, and to decide the best treatment. Areas covered: a) Epidemiology: BD is rare, and is seen along the Silk Road, from 20 to 420/100,000 in Turkey and 80/100,000 in Iran, to 0.

Adult Behcet's disease in Iran: analysis of 6075 patients.

Introduction: This is an analysis of adult Behcet's disease (BD) in Iran, from the Iran Registry of 7187 BD patients, gathered from 1975 to 2014, among which 6075 were adults (84.5%).

Patients: Patients were seen by a multidisciplinary team of experts.

The saga of diagnostic/classification criteria in Behcet's disease.

There are 17 sets of diagnosis/classification criteria for Behcet's disease: Curth (1946), Hewitt (1969), Mason (1971), Japan (1972), Hubault (1974), O'Duffy (1974), Cheng (1980), Dilsen (1986), Japan revised criteria (1988), International Study Group on Behcet's disease (ISG criteria, 1990), Iran traditional criteria (1993), Iran Classification Tree (1993), Dilsen revised criteria (2000), Korea Criteria (2003), International Criteria for Behcet's Disease (ICBD, 2006) and the revised ICBD (2010). This review is intended to show how to use them and show their performance in patients from different parts of the world. The major sets of patients (patient numbers, control numbers, year) on which the criteria were tested are: ISG set (886/97/1990), Iran (2069/1540/1993), Asia and Pacific League of Associations for Rheumatology (APLAR: 216/145/1998), Russia (105/233/2000), USA (50/NA/2000 [NA: not available]), India (50/NA/2004), Singapore (37/NA/2004), China (98/NA/2004), Korea (1454/NA/2004), Iran (4900/2020/2004), ICBD (2556/1163/2006), Germany (86/38/2008), China (322/118/2008), Iran (6128/3400/2010) and Iran (7011/5226/2013).

Combination of pulse cyclophosphamide and azathioprine in ocular manifestations of Behcet's disease: longitudinal study of up to 10 years.

Aim: Ocular lesions of Behcet's disease (BD) need aggressive treatment to prevent severe loss of vision or blindness. Cytotoxic drugs are the main therapeutic agents and the first line treatment. Retinal vasculitis is the most aggressive lesion of ocular manifestations and predicts a worse systemic outcome.

Clinical features of Behcet's disease in patients without oral aphthosis.

Objectives: In current study we evaluated clinical features of Behcet's Disease (BD) in patients without oral aphthosis (NOA cases).

Methods: In a cohort of BD, patients registered during a period of 36 years were collected. We determined clinical features of BD NOA cases and compared them with patients with oral aphthosis (OA cases).

Methotrexate in ocular manifestations of Behcet's disease: a longitudinal study up to 15 years.

Background: Ocular manifestations of Behcet's disease (BD) need aggressive treatment to prevent severe loss of vision or blindness. Cytotoxic drugs are the main therapeutic agents and the first line treatment. Methotrexate is the least toxic, used mainly for posterior uveitis.

Validation of the revised International Criteria for Behcet's Disease (ICBD) in Iran.

The first internationally agreed criteria for Behcet's disease were the International Study Group (ISG) criteria. It had very high specificity, but lacked good sensitivity, missing an important subset of patients. The International Criteria for Behcet's Disease (ICBD) were created in 2006 to overcome this lack of sensitivity.

Mesenchymal stem cell therapy unable to rescue the vision from advanced Behcet's disease retinal vasculitis: report of three patients.

Objective: Retinal vasculitis (RV) is the most aggressive lesion of ocular manifestations of Behcet's disease, seen in 32.1% of patients. Although visual acuity (VA) improves with early and aggressive treatment, in the long run it is seen in only 48% of patients.

Behcet's disease: is there a gender influence on clinical manifestations?

Aim:   In Behcet's disease (BD), it is customary to believe that men are more affected than women, major organs are more involved in men, and they have worse outcomes. The male-to-female ratio is reported from 5.37 to 1 (Egypt), to 0.

Impact of the positive pathergy test on the performance of classification/diagnosis criteria for Behcet's disease.

Background: The only diagnostic test that currently exists for Behcet's disease (BD) is the pathergy test. A positive pathergy test (PPT) is an important component of many of the 16 sets of classification/diagnosis criteria used to diagnose BD. The aim of this study was to determine the importance of a PTT in the performance of the diagnosis/classification criteria for BD.

Diagnostic value of pathergy test in Behcet's disease according to the change of incidence over the time.

Pathergy test (PT) is used for the diagnosis of Behcet's disease (BD). It is a criterion in many classification/diagnosis criteria. PT is mainly seen in BD but can be seen in other conditions too.

Behcet's disease in Iran: analysis of 6500 cases.

Objective: To identify the clinical picture of Behcet's disease in a large cohort of patients (6500) in Iran, over a period of 35 years, and compare them with other large series from around the world.

Methods: Patients with Behcet's disease from all over Iran were seen in the Behcet's Disease Research Unit by a multidisciplinary team (rheumatologists, dermatologists, and ophthalmologists). Diagnosis was based on 'expert opinion'.

Gender influence on ocular manifestations and their outcome in Behcet's Disease. A long-term follow-up of up to 20 years.

It is of general belief that males are prone to more frequent, more severe manifestations, and less favorable outcome. We evaluated this hypothesis in ophthalmological manifestations (OM) of Behcet's Disease (BD). Visual acuity (VA), anterior uveitis, posterior uveitis (PU), and retinal vasculitis (RV) were checked, according to Ben Ezra, in 1,515 patients with eye lesions.

Rituximab in intractable ocular lesions of Behcet's disease; randomized single-blind control study (pilot study).

Background: Ocular lesions, the main morbidity of Behcet's disease (BD), are the most difficult to treat. The aim of this study was to evaluate the efficacy of rituximab.

Methods: Inclusion criteria were retinal vasculitis and edema, resistant to cytotoxic drugs.

How to deal with Behcet's disease in daily practice.

Introduction: Behcet's Disease (BD) is classified as a vasculitis, and progresses via attacks and remissions. BD is mainly seen around the Silk Road. The picture varies in different reports.

Validation of the International Criteria for Behçet's disease (ICBD) in Iran.

Aim: To evaluate the performance of the new International Criteria for Behçet's Disease (ICBD) in Iran. The ICBD was created in 2006 to replace the International Study Group (ISG) criteria (created in 1990). In this study, 14 existing diagnosis/classification criteria (Curth, Hewitt, Mason and Barnes, Japan original, Hubault and Hamza, O'Duffy, Cheng and Zhang, Dilsen, Japan revised, International Study Group, Iran traditional, Iran Classification Tree, revised Dilsen, Korea), were evaluated and compared to ICBD by calculating their sensitivity, specificity and accuracy.

Behcet's disease: from East to West.

Behcet's disease (BD) is classified among vasculitides. There are actually five nationwide surveys of BD: Iran, Japan, China, Korea, and Germany. Among case series, four are on more than 200 cases (Turkey, Morocco, Tunisia, and UK).

Colchicine versus placebo in Behçet's disease: randomized, double-blind, controlled crossover trial.

The effect of colchicine was evaluated in a large cohort of Behçet's disease (BD) patients and compared to placebo. In a randomized, double-blind, controlled crossover trial, 169 patients without major organ involvement were selected consecutively. They fulfilled the International Criteria for Behçet's Disease.