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Behcets syndrome

Maria Zerkaoui, Fatima Zahra Laarabi, Yousra Ajhoun, Bouchra Chkirate, Abdelaziz Sefiani
BACKGROUND: Familial Mediterranean fever is an autoinflammatory disease of unknown etiology, characterized clinically by recurrent attacks of sudden-onset fever with arthralgia and/or thoracoabdominal pain and pathogenetically by autosomal recessive inheritance due to a mutation in the MEFV gene. Behçet's disease is an inflammatory disease characterized by recurrent oral and genital aphthous ulcerations, uveitis, and skin lesions. Preliminarily, our literature review suggested that patients with familial Mediterranean fever who also have Behçet's disease have only a single mutated familial Mediterranean fever gene...
March 1, 2018: Journal of Medical Case Reports
Sameer Lakha, C Y Maximilian Png, Kevin Chun, Windsor Ting
OBJECTIVE: Vascular manifestations including pulmonary artery aneurysms and venous thrombosis are seen in up to 14% of patients with Behcet's Disease. We report a patient who had recurrent deep vein thrombosis (DVT) as the presenting symptom of Behcet's Disease. METHODS: A 19-year-old male who presented with acute iliofemoral DVT, confirmed by intravascular ultrasound (IVUS) and venogram. May-Thurner syndrome was also observed. Repeated catheter-based pharmaco-mechanical thrombolysis, thrombectomy and subsequent iliac vein stenting were performed...
February 23, 2018: Annals of Vascular Surgery
Serkan Demirkan, Yıldırım Gültekin
Hughes-Stovin syndrome is a rare disorder of unknown etiology. Although the association between multiple pulmonary artery aneurysms and venous thrombosis of the lower limbs was reported by Beattie and Hall in 1911, it was not until 1962 that the eponym "Hughes-Stovin syndrome" was formally introduced in the medical literature. We describe 2 patients with Hughes-Stovin syndrome who presented with pulmonary artery aneurysm, thrombophlebitis, hemoptysis, and oral ulcers, review the manifestations of the disease, and compare its similarities with and differences from Behçet disease...
February 2018: Korean Journal of Thoracic and Cardiovascular Surgery
Minkyung Han, Yoon Suk Jung, Won Ho Kim, Jae Hee Cheon, Sohee Park
Background/Aims: The relationship between intestinal Behçet's disease (BD) and cancer remains unclear. We conducted a nationwide, population-based study to determine the risk of cancer in patients with intestinal BD. Methods: Using the National Health Insurance claims records, we collected data on 365 patients who had been diagnosed with intestinal BD between 2011 and 2014. Standardized incidence ratios (SIRs) of overall and site-specific cancers in patients with intestinal BD in comparison with the general population were calculated...
February 13, 2018: Gut and Liver
Yong Chen, Yan Shen, Hai-Fen Ma, Jian-Fei Cai, Yan-Qin Hua, Jun Zou, Jian-Long Guan
RATIONALE: Tumor necrosis factor (TNF-α) participates in the pathophysiology of Behcet's disease (BD) and myelodysplastic syndrome (MDS). Infliximab is recommaned for the most severe type of BD, however, there is little evidence for its effectiveness in BD associated MDS. PATIENT CONCERNS: A 46-year-old female, initially diagnosed with intestinal BD and leukopenia was later diagnosed as MDS. Treatement with infliximab and other immunoregulators lead to life-threatening pneumonia...
December 2017: Medicine (Baltimore)
Laurel L Wessman, Louise K Andersen, Mark D P Davis
Understanding the effects of age on the epidemiology of diseases primarily affecting the skin is important to the practice of dermatology, both for proper allocation of resources and for optimal patient-centered care. To fully appreciate the effect that age may have on the population-based calculations of incidence of diseases primarily affecting the skin in Olmsted County, Minnesota, and worldwide, we performed a review of all relevant Rochester Epidemiology Project-published data and compared them to similar reports in the worldwide English literature...
January 29, 2018: International Journal of Dermatology
Hasan Yazici, Emire Seyahi, Gulen Hatemi, Yusuf Yazici
This corrects the article DOI: 10.1038/nrrheum.2017.208.
January 24, 2018: Nature Reviews. Rheumatology
Seung-Shin Lee, Jae-Sook Ahn, Sook Jung Yun, Dong-Jin Park
No abstract text is available yet for this article.
December 2017: Blood Research
Pietro Maggi, Martina Absinta, Matteo Grammatico, Luisa Vuolo, Giacomo Emmi, Giovanna Carlucci, Gregorio Spagni, Alessandro Barilaro, Anna Maria Repice, Lorenzo Emmi, Domenico Prisco, Vittorio Martinelli, Roberta Scotti, Niloufar Sadeghi, Gaetano Perrotta, Pascal Sati, Bernard Dachy, Daniel S Reich, Massimo Filippi, Luca Massacesi
OBJECTIVES: In multiple sclerosis (MS), MRI is a sensitive tool for detecting white matter lesions, but its diagnostic specificity is still suboptimal; ambiguous cases are frequent in clinical practice. Detection of perivenular lesions in the brain (the "central vein sign") improves the pathological specificity of MS diagnosis, but comprehensive evaluation of this MRI biomarker in MS-mimicking inflammatory and/or autoimmune diseases, such as CNS inflammatory vasculopathies, is missing...
January 12, 2018: Annals of Neurology
Dilcan Kotan, Murat Alemdar, Semra Alaçam Köksal
No abstract text is available yet for this article.
December 2017: Noro Psikiyatri Arsivi
Hasan Yazici, Emire Seyahi, Gulen Hatemi, Yusuf Yazici
The presence of symptom clusters, regional differences in disease expression and similarities with, for example, Crohn's disease suggest multiple pathological pathways are involved in Behçet syndrome. These features also make formulating disease criteria difficult. Genetic studies have identified HLA-B*51 to be the important genetic risk factor. However, the low prevalence of HLA-B*51 in many patients with bone fide disease, especially in non-endemic regions, suggests other factors must also be operative in Behçet syndrome...
January 3, 2018: Nature Reviews. Rheumatology
Yoon Suk Jung, Minkyung Han, Do Young Kim, Jae Hee Cheon, Sohee Park
BACKGROUND: Various immune-mediated diseases are associated with increased malignancy risks. However, the relationship between Behçet's disease (BD) and cancer remains unclear. We conducted a nationwide, population-based study to determine the risk of cancer in patients with BD. METHODS: Using National Health Insurance claims records, we collected data from 2402 patients diagnosed with BD between 2013 and 2014. Standardized incidence ratios (SIRs) of overall and site-specific cancers in patients with BD in comparison with the general population were calculated...
2017: PloS One
Yan Jiang, Ling Cheng, Xin Li, Wenke Zhou, Li Zhang
The present study was designed to explore the interrelationship between single nucleotide polymorphisms (SNP) of the tumor necrosis factor superfamily (TNFSF) and its respective receptor superfamily (TNFRSF) genes and Behcet's disease (BD) and Vogt-Koyanagi-Harada syndrome (VKH) in Han Chinese. The study sample included 796 patients with BD, 792 patients with VKH syndrome, and 1604 healthy controls. The genotyping of 35 SNPs was performed by MassARRAY platform (Sequenom), iPLEX Gold Assay, PCR-restriction fragment length polymorphism assay and TaqMan SNP assay...
December 1, 2017: Oncotarget
Sila Seremet, Mehmet Salih Gurel
The link between the metabolic syndrome (MetS) and skin diseases is increasingly important, with new associations being discovered. The association between MetS and psoriasis or MetS and hidradenitis suppurativa is well known, although the relationship between MetS and various autoimmune or inflammatory diseases has only recently attracted interest. Some inflammatory skin diseases, such as vitiligo, scleredema, recurrent aphthous stomatitis, Behçet disease, rosacea, necrobiosis lipoidica, granuloma annulare, skin tags, knuckle pads, and eruptive xanthomas, have possible associations with MetS...
January 2018: Clinics in Dermatology
Stella Pak, Shaina Logemann, Christine Dee, Adam Fershko
Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome refers to a condition in which features of Behcet's disease (BD) and relapsing polychondritis (RP) occur in the same individual. The existence of MAGIC syndrome suggests a potential common etiology for BD and RP. However, connecting these two diseases and referring to this condition as MAGIC syndrome might have been premature, as there is currently insufficient knowledge on BD and RP. In this critical review, we argue that these two clinical entities could possibly be unique disease processes rather than two ends of the same disease spectrum...
October 4, 2017: Curēus
Melek Kechida, Sondes Yaacoubi, Ahmed Zrig, Walid Jomaa, Rim Klii, Sonia Hammami, Ines Khochtali
Background: Hughes-Stovin Syndrome (HSS) is a rare clinical disorder characterized by deep venous thrombosis and multiple pulmonary and/or bronchial aneurysms. Aneurysms in systemic circulation can also be seen. Case presentation: We report the first case of HSS with aortic aneurysm in a 55-year-old man who initially presented with deep venous thrombosis. The diagnosis of HSS revealing Behçet's disease was made given the history of recurrent oral and genital ulcers...
2017: Caspian Journal of Internal Medicine
Ghazaleh Mehdipoor, Fereydoun Davatchi, Hadi Ghoreishian, Abbas Arjmand Shabestari
Behcet's disease is an autoimmune disease most commonly seen in the Middle East. Although primarily known with painful oral and genital ulcers, it can lead to vasculitis. Therefore, several associated complications such as thrombotic syndromes, aneurysmal arterial disease may arise. In many cases, it might be difficult to make the diagnosis purely based on clinical grounds; however, imaging plays an important role for both diagnosis and assessment of the disease's complications. We provide a comprehensive review of the most notable imaging findings of Behcet's disease...
January 2018: European Journal of Radiology
Pietro Leccese, Immacolata Attolico, Angela Padula, Agostino Diplomatico, Salvatore D'Angelo, Ignazio Olivieri
No abstract text is available yet for this article.
November 2017: Clinical and Experimental Rheumatology
Gul Guzelant, Didar Ucar, Sinem Nihal Esatoglu, Gulen Hatemi, Yilmaz Ozyazgan, Sebahattin Yurdakul, Emire Seyahi, Hasan Yazici, Vedat Hamuryudan
OBJECTIVES: The prognosis of uveitis in Behçet's syndrome (BS) has improved over decades. Whether this is related to the use of more aggressive management strategies is not known. METHODS: This is a retrospective study of BS patients who received infliximab (IFX) for refractory eye disease between 2003-2015. The patients were divided into two groups according to the date of onset of in IFX treatment as before and after 2013. We compared the two groups in terms of disease characteristics at the onset of IFX treatment and response to treatment...
November 2017: Clinical and Experimental Rheumatology
Gul Guzelant, Yesim Ozguler, Sinem Nihal Esatoglu, Guzin Karatemiz, Huri Ozdogan, Sebahattin Yurdakul, Hasan Yazici, Emire Seyahi
OBECTIVES: Menstruation triggers several conditions such as migraine, recurrent aphthous stomatitis and acne vulgaris in healthy individuals. There is evidence that Behçet's syndrome (BS) and familial Mediterranean fever (FMF) may exacerbate during menstruation. The aim is to assess whether BS and FMF patients experience menstrual flares. METHODS: Females of reproductive age with BS and FMF seen consecutively at the outpatient clinic of Cerrahpasa Medical Faculty at Istanbul, as well as apparently healthy hospital workers were studied using a standardised questionnaire...
November 2017: Clinical and Experimental Rheumatology
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