scholarly journals Behcet’s Disease: Pakistani Experience

2020 ◽  
Vol 36 (5) ◽  
Author(s):  
Asadullah Khan ◽  
Muhammad Haroon ◽  
Farhan Bashir ◽  
Zia ud Din

Objective: To analyze the clinical manifestation of patients with Behcet’s disease, and performance of different classification criteria of Behcet’s disease in our population. Methods: It was a retrospective analysis of all Behcet’s disease patients attending Department of Rheumatology at Fatima Memorial Hospital, Lahore, Pakistan from April 2019 to July 2019. We performed a comprehensive clinical evaluation of patients with Behcet’s disease, with focus on patients’ age, gender and different clinical manifestations. Results: A consecutive cohort of 20 patients was studied. All patients met the International Criteria of Behcet’s Disease criteria, and 18 out of 20 patients also met International Study Group criteria. Mean age of the cohort was 33.5±10.4 years and 45% was female (male to female ratio of 1:1.2). Around 90% of cohort had recurrent oral and genital ulcers. Ocular involvement was present in 80% patients, while Joint manifestations were present in 75% of patients. Cutaneous, neurological (both central and peripheral nervous system involvement), and GIT symptoms were present in 50%, 30%, and 15% of patients, consecutively. Joint pain and eye symptoms were major initial symptoms in males, while eye symptoms and neurological symptoms were more common in female patients at disease onset. Reaching border line significance, Cutaneous (p-value=0.479), ocular (p-value=0.61), and GIT involvement (p-value=0.59) were more prevalent in males while neurological involvement (p-value=0.336) in females. Conclusion: Behcet’s disease occurs commonly in middle age population with equal male to female ratio, with mucocutaneus aphthosis, ocular disease and joint pains being common manifestation. Gastrointestinal symptoms are more common in males while neurological symptoms in females. doi: https://doi.org/10.12669/pjms.36.5.1916 How to cite this:Khan A, Haroon M, Bashir F, Zia-ud-Din. Behcet’s Disease: Pakistani Experience. Pak J Med Sci. 2020;36(5):---------. doi: https://doi.org/10.12669/pjms.36.5.1916 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1537.1-1537
Author(s):  
R. Goloeva ◽  
Z. Alekberova

Background:Behcet’s disease (BD) is systemic vasculitis, which affects all types and sizes of vessels. Increased carotid intima-media thickness (IMT) is parameter associated with subclinical atherosclerosis.Objectives:To determine the prevalence of atherosclerosis in pts with BD.Methods:95 BD pts were evaluated and 45 healthy controls matched for age and gender.IMT was assessed by high-resolution B-mode ultrasonography. Serum concentration of high-sensitivity C-reactive protein (hs CRP) was measured by immunonephelometric assay (BN-100 Analyzer; Dade Behring). Lipid profile evaluation included total cholesterol, TGs, HDL, LDL and atherogenic index.Results:The male-to-female ratio was 3,7:1, the mean age of pts was 29.7 (23-35) yrs, the mean age at the disease onset - 19,9 (14-25) yrs, the mean disease duration - 9,6 (4-15) yrs.Conclusion:Coronary atherosclerosis in BD pts was lower than what we expected. The thinning IMT may be one of the risk factors for aneurysm formation in pts with BD.Disclosure of Interests:None declared


Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
R Cherfi ◽  
N Mahieddine ◽  
K Mammeri ◽  
S Touri ◽  
F Sadaoui ◽  
...  

Abstract Background Behçet's disease is a rare systemic vasculitis in children. The most frequent manifestations are orogenitalaphthosis and uveitis. Neurological, digestive, vascular, joint, and skin involvement are possible. We aimed to describe the clinical, diagnostic criteria, and therapeutic features of pediatric-onset Behcet's disease Methods Study carried out based on files collected over 12 years (2007–2018) concerning patients under the age of 15 hospitalized in the pediatric department of CHU de Blida for Behcet disease Results For 12 years (2007–2018), we hospitalized five children for various reasons and whose diagnosis of Behcet's disease was retained. These are 03 boys (2 of the theme are brothers) and 02 girls. The average age at admission was 13.5 years (12–15 years), and the average duration of follow-up is 4.2 years (15 months-8 years). The mean age of onset of first symptoms was 11 years, and the meantime to diagnosis was 2 years (12 months-3 years) The main diagnostic criteria were: mouth ulcers in five patients (100%), genital ulcers in three patients (60%), ophthalmic manifestations in two patients (40%): one had bilateral uveitis, and the other had papillitis, the pathergy test was positive in 3 patients (60%). The other manifestations were as follows: polyarthralgia in 3 patients (60%), family history in one patient (20%). Neurological manifestations were present in 3 patients (60%): one child presented two episodes of aseptic meningitis and thrombosis deep cerebral venous; one child with diffuse cerebral demyelination lesions, and the third with HIC syndrome and massive lateral sinus thrombosis. Three children have a vascular involvement (60%): pulmonary embolism with thrombosis of the superior vena cava (20%), bilateral thrombosis of the jugular veins (20%), and thrombosis of the right ventricle complicated by pulmonary embolism (20%) Initial symptoms included recurrent aphthous stomatitis (100%), arthralgia (60%), prolonged fever (20%), behavioral disturbances and dysarthria (20%), weight loss (40%), and headache (60 %). The histocompatibility antigen (HLAB 51) was found in 2 children (40%). The treatment included corticosteroids for all patients (3 boluses of 1 g/m2/day of methylprednisolone then relayed by prednisone 1 mg/kg/day), azathioprine 2.5 mg/kg/day in 5 children (100%), and colchicine for 2 patients only one patient had received anticoagulant therapy (LMWH followed by VKA) Relapses were observed in a single patient (20%) and mortality in one patient (20%.) Conclusion Behcet's disease is a rare disease whose diagnosis remains difficult in children. However, early diagnosis and aggressive treatment are mandatory to avoid complications and functional sequelae.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1409.2-1409
Author(s):  
R. Goloeva ◽  
Z. Alekberova

Background:Early diagnosis and identification of predominant organ system involvement in Behcet’s disease (BD) is crucial for identifying most optimal treatment. The study evaluates 6 described phenotypes of BD - cutaneous-mucous, articular, ophthalmic, vascular, neurological and intestinal in different ethnic groups.Objectives:To assess the occurrence of Behcet’s disease (BD) phenotypes in different ethnic groups.Methods:The study included 202 patients with BD from the 5 most common ethnic groups.The male-female ratio was 2.4:1. Patients’ mean age was 31 years [24;37], mean age at the disease onset was 21 years [15;28]; and mean disease duration was 7 years [3;14]. The severity of BD (mild, moderate and severe) was assessed based on the I. Krause’s Clinical Severity Scoring for BD.Results:Severe BD was more often diagnosed in Azerbaijanis and indigenous residents of Dagestan compared to Russians (75 and 70.4% vs. 36.2%), in Armenians - 50% and Chechens - 54.5% out of all BD cases.Russians were significantly more likely to have a neurological phenotype (15.5% vs. 0-9.4% in all other ethnic groups) and intestinal phenotype (36.2% vs. 13.8-22.7 in all other ethnic groups). Azerbaijanis demonstrated higher prevalence of ocular involvement (68.7% versus 36.2% in Russians, 50% - in Chechens and Armenians, and 57% - in Dagestanis). Dagestanis were more likely to have a vascular phenotype (40.7% versus 15.6% in Azerbaijanis and 18.9% in Russians). The male/female ratio among Russian patients was 1:1, among Dagestanis 4.4:1, Azerbaijanis 3.5:1, Chechens and Armenians 2.6: 1.Conclusion:BD phenotypes vary and demonstrate significant association with the patient’s ethnic affiliation therefore, ethnicity should be viewed as the prognostic marker of specific organ-system involvement in case of a disease.Disclosure of Interests:None declared


VASA ◽  
2018 ◽  
Vol 47 (5) ◽  
pp. 427-430 ◽  
Author(s):  
Barbara Geeroms ◽  
Philippe Demaerel ◽  
Joost Wauters ◽  
Johny Verschakelen ◽  
Geert Maleux

Abstract. Onset of neurological symptoms early after intranodal lymphangiography can occur due to Lipiodol droplet migration through intrapulmonary lymphovenous communication. Patients with Behçet’s disease may be at higher risk of developing this devastating complication.


Reumatismo ◽  
2020 ◽  
Vol 71 (4) ◽  
pp. 218-225
Author(s):  
D.H.S. Attia ◽  
R.A. Abdel Noor

Behçet’s disease is a relapsing multisystemic disease. Its highest prevalence is seen along the Silk Road. While several studies reported gender disparities, others didn’t. Scarce data are available about the Arabs and the gender differences detected in some ethnicities could not be applied to others. Our study aimed to detect gender differences among a cohort of adult Egyptian patients with Behçet’s disease. Medical files of 255 adult patients diagnosed with Behçet’s disease at the Rheumatology and Rehabilitation Department of Kasr Al-Aini Hospital, Cairo University and the Internal Medicine Department of Tanta University, between 2002 and 2018, were retrospectively reviewed. The demographic features, the cumulative clinical features, the use, if any, of an intravenous pulse of methylprednisolone and immunosuppressive/biological drugs, and the frequency of the cumulative damage to the different organ systems were described. The disease severity score was calculated as well. The study revealed a prominent male predominance; the male to female ratio was 6.7:1. Acne/pseudofolliculitis was more common in males (28.6% vs 13.2%, p=0.046); the same was observed regarding any vascular involvement and peripheral venous disease (36.3% vs 18.4%, p=0.03 and 30.4% vs 13.2%, p=0.03, respectively). On the other hand, encephalitis and cranial nerve lesions were more prevalent in females (15.8% vs 6%, p=0.03 and 10.5% vs 3.2%, p=0.04, respectively). Severe Behçet’s disease equally affects Egyptian males and females. BD should not be considered a benign disease in Egyptian females. The same quality of medical care should be equally offered to both genders.


2021 ◽  
Author(s):  
Soraya Shadmanfar ◽  
Maryam Masoumi ◽  
Fereydoun Davatchi ◽  
Farhad Shahram ◽  
Maassoumeh Akhlaghi ◽  
...  

Abstract BackgroundBehçet’s disease (BD) is a chronic disorder that involves multiple organs and is pathologically considered as a form of vasculitis. The current study aims to assess the metric properties of platelet to lymphocyte ratio (PLR) and neutrophil to lymphocyte ratio (NLR) in assessing BD disease activity.MethodsThree-hundred-nineteen patients with BD were enrolled in this cross-sectional study. Demographic and epidemiological data, including IBDDAM, time since the onset, and medication and manifestation history was recorded. Complete blood counts (CBC), NLR, and PLR were assessed by analyzing blood samples. On the last visit, patients were assessed for active manifestations of disease. IBDDAM and ocular IBDAAM scores were calculated for activity of disease in each patient.ResultsBoth PLR and NLR were higher in patients with active BD (Man-Whitney U test, P-value<0.05). Patients with active ocular manifestation had significantly higher NLR and PLR (Man-Whitney U test, P-value<0.05). These ratios, however, were not associated with other active BD manifestations. A value of NLR >2.58 had 46% sensitivity and 85% specificity for the diagnosis of active ocular manifestations (AUC: 0.690). NLR had a significant though weak positive correlation with IBDDAM (Spearman’s rho = 0.162; p-value <0.05) and ocular IBDDAM (Spearman’s rho = 0.159; p-value < 0.05). ConclusionActive Behçet’s presented with higher NLR and PLR ratios; however, there was only a modest correlation between NLR and BD activity (IBDDAM score). Also NLR and PLR have significant relationship with ocular features of BD patients.


2013 ◽  
Vol 94 (4) ◽  
pp. 545-548
Author(s):  
R F Khamitov ◽  
L Y Palmova ◽  
Z N Yakupova ◽  
E R Khasanova

Behçet’s disease is a chronic relapsing multisystemic vasculitis involving vessels of different diameter. The disease is characterized by involvement of skin and mucosa, eyes, gastrointestinal tract, joints, vessels, genitourinary system and neurological symptoms. According to data, male/female ratio is 2-10:1 The etiology of the disease is still unclear. Diagnosis is mainly based on thorough analysis of clinical manifestations. According to the International Study Group for Behçet’s Disease Diagnostic Criteria, combination of recurrent oral aphthosis and at least any two of the following: severe or scarring genital aphthosis, eye lesions (posterior uveitis, retinal vasculitis), skin lesions (nodular erythema, pseudofolliculitis, acne-like lesions) and positive pathergy test is needed to classify the patient as a patient with Behçet’s disease. Several clinical subtypes of Behçet’s disease are distinguished depending on predominant involvement of different organs and systems, including skin and mucosa, joints, eyes, or neurological manifestations. There are no specific laboratory tests for Behçet’s disease. The treatment approaches are variable depending on clinical manifestations. The effect of systemic corticosteroids, colchicine, azathioprine, chlorambucil, cyclophosphamide, sulfasalazine, methotrexate, cyclosporine, infliximab was proven. All the abovementioned requires the good clinical knowledge on Behçet’s disease from practicing doctors of different specialties, particularly - physicians. A case of highly active acute Behçet’s disease with nose, pharynx, eye and oral cavity mucosa, scrotum skin involvement is presented in the article. Questions of differential diagnosis are reviewed, specific treatment program with efficacy analysis is presented.


2019 ◽  
Author(s):  
Hao Yin ◽  
Yun Song ◽  
Meimei Zheng ◽  
Han Ju ◽  
Na Shao ◽  
...  

Abstract Background Behcet’s disease (BD) is a multi-systemic vasculitis which generally characterized by oral and genital ulcers as well as ocular and skin lesions. There are only 5% of BD patients get neurological involvement and it usually occurs 4-6 years later after the initial symptoms. Early onset of neurological impairment symptoms makes it difficult to diagnose and treat definitely. Case presentation A 38-year-old man with BD was referred to our hospital due to a sudden numbness and weakness in his left extremities. Brain magnetic resonance imagine (MRI) showed acute cerebral infarction. The symptom was recurred in several months and then there were skin rash, oral ulcer and arthrodynia. High resolution MRI of brain suggested cerebral artery intima thickening. Laboratory examination showed the increase of IL-8. Biopsy of the skin rash showed small vessel vasculitis. We treated the patient with an immunosuppressive agent mycophenolate mofetil (MMF), hydroxychloroquine and colchicine. Now, patients still insist on taking immunosuppressive agents, all symptoms were alleviated after treatment, rash decreased in the past six months, no acute stroke, no recurrence of oral ulcer. Conclusion This case demonstrates that neurological involvement might be the first symptom of Behcet’s disease, and IL-8 could be a useful marker for diagnosis.


Author(s):  
Mira Merashli ◽  
Tommaso Bucci ◽  
Daniele Pastori ◽  
Pasquale Pignatelli ◽  
Alessia Arcaro ◽  
...  

Abstract Aim To evaluate the relevance of plasma homocysteine (HC) in Behcet's disease (BD) and its clinical manifestations. Methods Systematic review of EMBASE and PubMed databases according to PRISMA guidelines from inception to July 2021; random-effects meta-analyses for continuous outcomes. Results The search strategy retrieved 48 case–control (2,669 BD and 2,245 control participants) and 5 cohort studies (708 BD participants). Plasma HC was higher in BD than in controls (p < 0.0001) with wide heterogeneity (I2  = 89.7%) that remained unchanged after sensitivity analysis according to year of article publication, age of BD participants, study size, study quality, method of HC determination, and male/female ratio >1.5; some pooled ethnicities explained a small part of the heterogeneity (I2  = 16.3%). Active BD participants had higher HC than inactive ones (p < 0.0001), with moderate heterogeneity (I2  = 49.2%) that disappeared after removal of an outlier study with very high disease activity. BD participants with any vascular involvement had higher HC than those without (p < 0.0001) with wide heterogeneity (I2  = 89.7%); subgroup analysis on venous thrombosis only changed neither effect size (p < 0.0001) nor heterogeneity (I2  = 72.7%). BD participants with ocular involvement had higher HC than those without (p < 0.0001) with moderate heterogeneity (I2  = 40.3%). Conclusion Although causality cannot be inferred, the consistency of the elevation of plasma HC in BD, particularly in patients with active disease, with vascular and ocular involvement suggests an intrinsic involvement of HC in these clinical manifestations.


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