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

Abstract

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. A BD diagnosis was confirmed (BD group) or ruled out (Non-BD group), and the two groups were compared for differences. Results A 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. Conclusions Being 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.

Case Report: A Case of Intestinal Behçet's Disease Exhibiting Enhanced Expression of IL-6 and Forkhead Box P3 mRNA After Treatment With Infliximab

“…Here, we report a case of intestinal BD that was successfully treated with IFX. This case was diagnosed as intestinal BD according to the international diagnostic criteria for BD (2). Our qPCR analyses of colonic biopsy samples revealed that the colonic mucosa of this patient was characterized by increased expression levels of IL-17, IL-23, TNF-α, IFN-β, CXCL9, and CXCL10 before treatment.…”

“…These endoscopic and pathological findings were not consistent with Crohn's disease or ulcerative colitis. Based on the presence of uveitis, oral ulcers, and intestinal symptoms, the patient was diagnosed with intestinal BD according to the international diagnostic criteria for BD (2).…”

“…Behçet's disease (BD) is a rare inflammatory condition characterized by oral and genital ulcers, skin lesions, and patients with BD exhibit ophthalmological, neurological, and gastrointestinal manifestations (1). Approximately 5-15% of patients with BD are diagnosed with intestinal BD, and they usually present with abdominal pain and diarrhea (1,2). A typical endoscopic finding in patients with intestinal BD is a giant, oval-shaped, deep punched-out ulcer in the ileocecal area (1).…”

Epidemiology of musculoskeletal symptoms, rheumatologic disorders, and disability in the Zoroastrian population in Yazd, Iran: a WHO-ILAR COPCORD study (stage 1)

“…Previous COPCORD studies performed in Iran have shown a 30–50% prevalence of musculoskeletal complaints, 15–25% for degenerative joint diseases, and approximately 1% for rheumatologic disorders [ 6 , 11 , 12 ]. Therefore, with a 5% type I error and an absolute error of 5%, this study would need > 138 individuals for a disease with 10% prevalence (e.g., musculoskeletal complaints), and > 1382 individuals for 1% prevalence (e.g., rheumatologic diagnoses).…”

Evaluation of diagnostic performance of haematological parameters in Behçet’s disease

“…BD begins between the ages of 20 and 40, but early and late onsets have been reported in some studies. 1 Because of the lack of pathognomonic laboratory tests, the diagnosis is based on related clinical findings. The International Working Group (ISG) diagnostic criteria, which published in 1990, continue to be the most widely accepted criteria among experts in BD.…”