Diagnosis of Beh\xe7et\u2019s disease: clinical characteristics, diagnostic criteria, and differential diagnoses

Mina Kiafar,Seyedeh Zahra Ghodsi,Azarakhsh Baghdadi,Farhad Shahram,Mohammad Nejadhosseinian,Seyedeh Tahereh Faezi,Seyed Ali Mousavi,Hormoz Shams,Fereydoun Davatchi,Sahar Kakaei,Amir Kasaeian

doi:10.1186/s41927-020-00172-1

Mina Kiafar, Seyedeh Zahra Ghodsi + Show 9 more

Open Access

https://doi.org/10.1186/s41927-020-00172-1

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Abstract

BackgroundThe 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.MethodsIn a retrospective study, we collected data from patients at a national referral Behçet clinic from November 2018–August 2019. A BD diagnosis was confirmed (BD group) or ruled out (Non-BD group), and the two groups were compared for differences.ResultsA total of 238 patients satisfied the inclusion criteria. Forty patients (16.8%) were finally diagnosed with BD. Ocular and genital lesions were significantly more prevalent in the BD group. A positive pathergy test and HLA-B51 were also significantly more common in BD. However, oral lesions, articular involvement, and gastrointestinal manifestations were similar between groups. Also, patients with BD were significantly more likely to have multi-organ (≥2 organ systems) involvement.ConclusionsBeing the first study to evaluate the clinical characteristics of patients who are visited at a referral BD clinic and are believed to have a high probability of Behçet, the results of this study are important from an epidemiological standpoint. Also, the findings of this study could be used by referral Behçet clinics, which evaluate and diagnose patients with a high pretest probability and atypical presentations of BD on a daily basis. The alternative diagnoses established in this study could be used as the list of the most common differential diagnoses for Behçet’s disease.

Highlights

The diagnosis of Behçet disease (BD) is challenging in many cases
In a retrospective cohort study, data from patients referred to the Behçet clinic of the Rheumatology Research Center (RRC) at Tehran University of Medical Sciences were reviewed from November 2018 to August 2019
We tried to answer the following question: when faced with a population of patients with a high probability of BD, which features are most predictive of a Behçet disease diagnosis? To the best of our knowledge, this is the first study with this novel approach, which was only made possible by being the single nationally-renowned referral Behçet clinic in the country

Summary

Introduction

The diagnosis of Behçet disease (BD) is challenging in many cases. Behçet disease (BD) is an inflammatory vasculopathy with multisystemic involvement. The clinical course usually follows a relapsing-remitting course with heterogeneous clinical manifestations [1]. Despite extensive research dedicated to the underlying mechanisms of BD, we still have a long way to understand the complexity of Behçet disease. Behçet disease has been reported all over the world, but the prevalence is high in the middle east, far east, and the Mediterranean. BD is referred to as the ‘silk route disease’, acknowledging the fact that the highest incidence of BD has been reported along this ancient route. Turkey has the highest prevalence of BD, followed by Iran, Saudi Arabia, Iraq, Israel, northern China, and Korea [2, 3]

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