Behcet’s Disease: Is There Geographical Variation? A Review Far from the Silk Road

Behcet’s Disease (BD) is a systemic vasculitis characterized by the triad of recurrent mouth and genital ulcers with eye involvement. To date there are no laboratory tests specific for the disease and diagnosis continues to remain on clinical grounds. Multiple criteria have been created as guides for diagnosis; however, given the wide spectrum of organ involvement, some cases remain undiagnosed. The diagnosis of Behcet’s Disease may only be made over time as the clinical manifestations emerge sometimes separated by months and even years. With an increased recognition of this disease it has become apparent that there is geographical variation in clinical manifestations. In particular cardiac manifestations are not seen commonly in Caucasians compared to Asian and Middle Eastern patients, while neurological manifestations are more common in Caucasians. Use of immunosuppressive and immunomodulatory drugs to suppress inflammation remains the cornerstone of treatment.

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Intestinal manifestations of Behçet's disease

Terapevticheskii arkhiv ◽

10.26442/00403660.2019.05.000247 ◽

2019 ◽

Vol 91 (5) ◽

pp. 111-119 ◽

Cited By ~ 1

Author(s):

R G Goloeva ◽

Z S Alekberova ◽

T A Lisitsyna ◽

E A Stepanova ◽

L P Orlova

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Systemic Vasculitis ◽

Clinical Manifestations ◽

Surgical Care ◽

Unknown Origin ◽

Massive Bleeding ◽

Fistula Formation ◽

Behcet's Disease

Behçet's disease (BD) is a systemic vasculitis of unknown origin, characterized by recurrences of the ulcerative process in the oral cavity and on the genitals, inflammatory damage of the eyes, joints, vessels and other organs. The severity and prognosis of BD determines organ pathology. Intestinal manifestations of BD (intestinal BD) are the least studied. Its verification in BD is complicated by the variety of clinical manifestations, their similarity with inflammatory bowel diseases, the lack of informative laboratory tests, pathognomonic endoscopic and histological signs. Intestinal BD can lead to serious complications (massive bleeding, intestinal perforation and fistula formation), which can not only significantly reduce the quality of the patient’s life, but also cause death. Treatment of intestinal BD is not standardized; it is mainly empirical and conducted courses. The purpose of therapy is to achieve clinical remission, healing of intestinal ulcers and prevention of surgery. The article presents a case of severe refractory intestinal BD, requiring twice emergency surgical care - removal of half and then the whole of the colon because of multiple perforations. A brief review of the literature is given and diagnostic difficulties of intestinal BD are discussed.

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Vanishing aneurysms in Behcet’s disease - A case series

Monaldi Archives for Chest Disease ◽

10.4081/monaldi.2021.1451 ◽

2021 ◽

Vol 91 (1) ◽

Author(s):

Taruna Yadav ◽

Suvinay Saxena ◽

Naveen Dutt ◽

Pawan Garg ◽

Pushpinder Khera

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Wide Spectrum ◽

Systemic Vasculitis ◽

Case Series ◽

Significant Treatment ◽

Mortality And Morbidity ◽

Behcet's Disease ◽

Pulmonary Arterial

Behçet’s disease is a systemic vasculitis with a wide spectrum of manifestations. Although pulmonary involvement is typical, the presence of pulmonary arterial aneurysms is associated with a high incidence of mortality and morbidity. We report two cases of Behçet’s disease with pulmonary arterial involvement who presented with hemoptysis, showed significant treatment response, thus, highlighting the importance of early diagnosis and therapeutic management.

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Expression of miR-146a and miR-155 in Egyptian patients with Behçet’s disease: clinical significance and relationship with disease activity

Egyptian Journal of Medical Human Genetics ◽

10.1186/s43042-020-00085-1 ◽

2020 ◽

Vol 21 (1) ◽

Author(s):

Engy El Khateeb ◽

Ayatallah Nassef ◽

Rasha Gheith ◽

Aya Erfan ◽

Walaa Abdelfattah

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Inflammatory Diseases ◽

Systemic Vasculitis ◽

Clinical Manifestations ◽

Expression Level ◽

Healthy Controls ◽

Stem Loop ◽

Behcet's Disease

Abstract Background Behçet’s disease (BD) is a systemic vasculitis disorder with multifactorial immunopathogenesis and associated with significant morbidity and mortality. MicroRNAs (miRNAs) are involved in the pathogenesis of inflammatory diseases. MiR-146 and miR-155 are known key regulators of immune response. This study was conducted to determine the expression of miRNA-146a and miRNA-155 in patients with BD and to link their possible association with the clinical manifestations and activity of this disease to evaluate their role as diagnostic or prognostic markers. A total of 60 patients with BD and 25 age- and gender-matched healthy controls were examined in a case-control study from October 2017 to September 2018 for the expression levels of miR-146a and miR-155 using singleplexTaqMan two-step stem loop quantitative reverse transcription real-time polymerase chain reaction (qRT-PCR). Results Patients with BD had significantly lower miR-146a levels than control subjects (P < 0.001). Regarding the miR-155 expression level, no statistically significant differences were detected between patients and healthy controls (P = 0.736). The expression level of miR-146a showed no significant association with the different clinical manifestations of patients with BD. Conclusion This study suggests the possibility that miR-146a expression in patients with BD is involved in the pathogenesis of disease. Furthermore, it can be used as a diagnostic biomarker and a therapeutic target for BD in the future.

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Old and New Challenges in Uveitis Associated with Behçet’s Disease

Journal of Clinical Medicine ◽

10.3390/jcm10112318 ◽

2021 ◽

Vol 10 (11) ◽

pp. 2318

Author(s):

Julie Gueudry ◽

Mathilde Leclercq ◽

David Saadoun ◽

Bahram Bodaghi

Keyword(s):

Behçet’S Disease ◽

Structural Damage ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Systemic Vasculitis ◽

Retinal Vasculitis ◽

Unknown Origin ◽

Current Data ◽

Ocular Involvement ◽

Behcet's Disease

Behçet’s disease (BD) is a systemic vasculitis disease of unknown origin occurring in young people, which can be venous, arterial or both, classically occlusive. Ocular involvement is particularly frequent and severe; vascular occlusion secondary to retinal vasculitis may lead to rapid and severe loss of vision. Biologics have transformed the management of intraocular inflammation. However, the diagnosis of BD is still a major challenge. In the absence of a reliable biological marker, diagnosis is based on clinical diagnostic criteria and may be delayed after the appearance of the onset sign. However, therapeutic management of BD needs to be introduced early in order to control inflammation, to preserve visual function and to limit irreversible structural damage. The aim of this review is to provide current data on how innovations in clinical evaluation, investigations and treatments were able to improve the prognosis of uveitis associated with BD.

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Behcet’s Disease: An In-depth Review About Pathogenesis, Gastrointestinal Manifestations And Management

Inflammatory Intestinal Diseases ◽

10.1159/000520696 ◽

2021 ◽

Author(s):

Anthony Nguyen ◽

Shubhra Upadhyay ◽

Muhammad Ali Javaid ◽

Abdul Moiz Qureshi ◽

Shahan Haseeb ◽

...

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Age Groups ◽

Clinical Manifestations ◽

Signs And Symptoms ◽

Vascular Disorder ◽

Robust Algorithms ◽

Behcet's Disease ◽

Relapsing Remitting

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.

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Pathergy Phenomenon

Frontiers in Medicine ◽

10.3389/fmed.2021.639404 ◽

2021 ◽

Vol 8 ◽

Author(s):

Tulin Ergun

Keyword(s):

Behçet’S Disease ◽

Complex Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Middle Eastern ◽

Adaptive Immune System ◽

Related Factors ◽

Behcet's Disease ◽

Sterile Needle ◽

Needle Prick

Skin pathergy reaction (SPR) is a hyperreactivity response to needle induced trauma which is characterized by a papule or pustule formation, 24–48 h after sterile-needle prick. It is affected by a wide array of factors, including the physical properties of the needles being used, number of pricks and disease related factors such as male gender, active disease. There is a great variation in reactivity among different populations with very low positivity rate in regions of low prevalence like Northern Europe, and higher prevalance mainly in communities living along the historical Silk Road, like Turkey, China and Middle Eastern countries. SPR is not constant during the disease course, has lost its sensitivity over decades and can be positive in various disorders including Sweet's syndrome, pyoderma gangrenosum, Crohn's diesease, A20 haploinsufficiency, deficiency of IL-1-receptor antagonist and few others. Nevertheless, it is a criteria included into many currently used diagnostic or classification criteria for Behçet's disease. Although, not being fully uncovered yet, available data points to the activation of both innate and adaptive immune system with an inflammatory response mediated by polymorphonuclears and T-cells. In addition to its utility in diagnosis of Behçet's Disease, SPR may serve as a model for investigating the inflammatory pathways involved in the etiopathogenesis of this complex disease.

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Acute Agitation as an Initial Manifestation of Neuro-Behçet’s Disease

Case Reports in Emergency Medicine ◽

10.1155/2018/5437027 ◽

2018 ◽

Vol 2018 ◽

pp. 1-3

Author(s):

Yuki Otsuka ◽

Tetsuya Yumoto ◽

Hiromi Ihoriya ◽

Namiko Matsumoto ◽

Kota Sato ◽

...

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Parietal Lobe ◽

Psychiatric Symptoms ◽

Behçet's Disease ◽

Physical Restraint ◽

Clinical Manifestations ◽

Initial Manifestation ◽

Behcet's Disease ◽

Acute Agitation

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.

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