Association of NOD1, NOD2, PYDC1 and PYDC2 genes with Behcet’s disease susceptibility and clinical manifestations

2021 ◽  
pp. 1-7
Author(s):  
Ayca Kocaaga ◽  
Gunes Cakmak Genc ◽  
Sevim Karakas Celık ◽  
Rafet Koca ◽  
Ahmet Dursun
Author(s):  
Anthony Nguyen ◽  
Shubhra Upadhyay ◽  
Muhammad Ali Javaid ◽  
Abdul Moiz Qureshi ◽  
Shahan Haseeb ◽  
...  

Background: Behcet’s Disease (BD) is a complex inflammatory vascular disorder that follows a relapsing-remitting course with diverse clinical manifestations. The prevalence of the disease varies throughout the globe and targets different age groups. There are many variations of BD, however, intestinal BD is not only more common but has many signs and symptoms. Summary: BD is a relapsing-remitting inflammatory vascular disorder with multiple system involvement, affecting vessels of all types and sizes that targets young adults. The etiology of BD is unknown but many factors including genetic mechanisms, vascular changes, hypercoagulability and dysregulation of immune function are believed to be responsible. BD usually presents with signs and symptoms of ulcerative disease of the small intestine; endoscopy being consistent with the clinical manifestations. The mainstay of treatment depends upon the severity of the disease. Corticosteroids are recommended for severe forms of the disease and aminosalicylic acids are used in maintaining remission in mild to moderate forms of the disease. Key messages: In this review, we have tried to summarize in the present review the clinical manifestations, differential diagnoses and management of intestinal BD. Hopefully, this review will enable health policymakers to ponder over establishing clear endpoints for treatment, surveillance investigations and creating robust algorithms.


2018 ◽  
Vol 2018 ◽  
pp. 1-3
Author(s):  
Yuki Otsuka ◽  
Tetsuya Yumoto ◽  
Hiromi Ihoriya ◽  
Namiko Matsumoto ◽  
Kota Sato ◽  
...  

Managing acutely agitated or violent patients in the emergency department (ED) represents a significant challenge. Acute agitation as an initial manifestation of neuro-Behcet’s disease (NBD) is an extremely rare clinical entity. A 44-year-old male, who had been complaining about a severe headache and fever for several days, was admitted to our ED due to acutely presented incontinence and agitation. On admission, physical restraint and sedation with sevoflurane and propofol were required for his combative and violent behavior. Cerebrospinal fluid examination revealed increased cell count. Fluid attenuated inversion recovery magnetic resonance imaging showed a high intensity signal in the left parietal lobe and bilateral occipital lobe. As infectious meningoencephalitis was suspected, empirical therapy was immediately started. He recovered uneventfully without neurological defect in seven days. Based on positive human leukocyte antigen B-51 and clinical manifestations, the diagnosis of NBD was made and remitted by steroid therapy. Although acute NBD commonly presents with focal neurological symptoms, psychiatric symptoms could be considered the first manifestation. A focused and thorough examination coupled with appropriate management strategies can assist emergency clinicians safely and effectively manage these patients.


2017 ◽  
Vol 1 ◽  
pp. 15-20 ◽  
Author(s):  
Nurşad Aslan ◽  
Kamile Demirci ◽  
Tuba Güler ◽  
Fulya Dörtbaş ◽  
Ebru Kale

2011 ◽  
Vol 68 (11) ◽  
pp. 992-995
Author(s):  
Aleksandra Tomic-Lucic ◽  
Snezana Jovanovic ◽  
Milan Petronijevic ◽  
Mirjana Veselinovic

Introduction. Behcet's disease is multisystemic vasculitis which affects vein and artery blood vessels. Intestinal perforation rarely occurs as clinic manifestation in as litle as 1% of patients. The transverse colon is the most infrequent site of perforation. We presented a patient diagnosed with Behcet's disease who underwent both surgical and conversative treatment due to perforation of the colon. Case report. A 34-year-old patient was admitted to the hospital with fever, aphthous ulcerations on oral mucosa and genitals and bilateral uveitis. On the basis of clinical symptoms and the International Criteria developed in 1990 Behcet's disease was diagnosed. During the next few days the patient developed erythema nodosum, diarrheic syndrome and acute abdominal symptoms due to perforation of the transverse colon. An emergent laparotomy was undertaken involving resection of a perforated segment of the colon, and bipolar colostomy on the left side of abdomen. Following the surgery the patient was treated except for antibiotics with three successive pulse doses of methylprednisolone (500 mg/daily) and cyclophosphamide (15 mg/kg). The treatment was continued by gradual decrease in the close of the corticosteroid (perorally) and by cyclophosphamide first with monthly doses (5 monthly pulse doses of 15 mg/kg cyclophosphoamide), and then with 3-month doses (totally 6 doses) up to totally 12 g. Conclusion. The therapy with pulse doses of methylprednisolone combined with pulse doses of cyclophosphamide was very effective in the reported case with the complex clinical manifestations leading to resolution of gastrointenstinal, ocular and orogenital lesions.


2011 ◽  
Vol 15 (3) ◽  
pp. 306-314 ◽  
Author(s):  
Fereydoun DAVATCHI ◽  
Farhad SHAHRAM ◽  
Cheyda CHAMS-DAVATCHI ◽  
Bahar SADEGHI ABDOLLAHI ◽  
Hormoz SHAMS ◽  
...  

2016 ◽  
Vol 7 (1) ◽  
pp. 141-147 ◽  
Author(s):  
Roy Schwartz ◽  
Sara Borok ◽  
Michaella Goldstein ◽  
Anat Kesler ◽  
Keren Regev ◽  
...  

Purpose: To report a unique case of Behçet's disease that presented with atypical ocular manifestations. Methods: Case report. Results: A 23-year-old homosexual male presented with bilateral anterior uveitis, vitritis, neuroretinitis and a unilateral superior hemivein occlusion with frosted branch angiitis pattern. These were accompanied by systemic findings of recurrent oral aphthous ulcers, erythema nodosum, and neurological and gastrointestinal involvement. A positive HLA-B51 examination supported the diagnosis of Behçet's disease. Conclusion: Neuroretinitis and frosted branch angiitis may be the clinical manifestations of Behçet's disease and may present simultaneously.


Reumatismo ◽  
2016 ◽  
Vol 67 (4) ◽  
pp. 156 ◽  
Author(s):  
M. S. Khanfir ◽  
N. Belfeki ◽  
F. Said ◽  
T. Ben Salem ◽  
I. Ben Ghorbel ◽  
...  

Inflammatory optic neuropathy (ON) is a rare event in Behçet’s disease (BD). We report herein a series of ten BD Tunisian patients with ON and describe its clinical features among them. A retrospective review of BD patients (International Study Group for BD criteria) was performed. The patients were divided into two groups: those presenting an inflammatory ON, and those none. The diagnosis of inflammatory ON was based on the clinical examination, visual field and visual evoked potentials. We analyzed the characteristics of the two groups. Ten patients (2.3%) presented an inflammatory ON among our 440 patients. Inflammatory ON was inaugural in 8 cases. Clinical manifestations were as follows: blurred vision (7 cases) and periorbital pain (3 cases). In two cases, the patients did not complain from ophthalmological symptoms. The fundus revealed a papilledema (2 cases), papillary pallor (4 cases), and was normal in 5 cases. Visual field realized in only three patients showed a scotoma in all cases. Visual evoked potentials revealed increased latency in all cases. All patients received corticosteroids associated to an immunosuppressive agent. The comparative study between the two groups revealed that inflammatory ON was significantly more associated to neurological involvement (p<0.0001) and that the disease was more severe in the ON group (p<0.0001). Inflammatory ON in BD is rare and may occur at an early stage of the clinical course of the disease. Its prevalence is certainly underestimated. A systematic visual evoked potential may be interesting as a screening tool.


2016 ◽  
Vol 13 (1) ◽  
pp. 57-65 ◽  
Author(s):  
Fereydoun Davatchi ◽  
Cheyda Chams-Davatchi ◽  
Hormoz Shams ◽  
Farhad Shahram ◽  
Abdolhadi Nadji ◽  
...  

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