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Intern Med 2019 Apr 17. Epub 2019 Apr 17.

Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Japan.

Autoimmune diseases including inflammatory bowel disease (IBD) occur in association with myelodysplastic syndrome (MDS). MDS-associated IBD frequently demonstrates a complicated course. We herein report the first case with MDS-associated IBD that was successfully treated with ustekinumab (UST), an anti-interleukin (IL) 12/23p40 monoclonal antibody. Read More

Brain Nerve 2019 Apr;71(4):329-333

Department of Dermatology, Saitama Medical University.

Behçet disease is an inflammatory disease of unknown etiology that affects various organs, such as the skin, eye, central nervous system, and blood vessels. Mucocutaneous manifestations are characterized by oral ulcers, genital ulcers, erythema nodosum, papulopustular eruptions, and thrombophlebitis. The diagnosis is based on the diagnostic criteria defined by the Japanese Ministry of Health, Labour and Welfare. Read More

Curr Rheumatol Rev 2019 Apr 15. Epub 2019 Apr 15.

Department of Immunology. Hospital Universitario Ramón y Cajal, Ctra. Colmenar Viejo, Km 9,100. C.P. 28034. Madrid. Spain.

Background: Behçet's Disease (BD, OMIM 109650) is a chronic relapsing inflammatory disease of unknown etiology with unpredictable exacerbations and remissions. First described in 1937 by the Turkish dermatologist Hulusi Behçet[1] as a trisympton complex (oral and genital ulcers and uveitis), it is now recognized as a multisystemic disease. The syndrome can manifest in diverse ways and can involve nearly every organ system. Read More

Int J Rheum Dis 2019 Apr 15. Epub 2019 Apr 15.

Department of Radiology, Diskapi Yildirim Beyazit Training and Research Hospital, University of Health Sciences, Ankara, Turkey.

Aim: We aimed to evaluate subclinical atherosclerosis based on carotid intima-media thickness (CIMT) and arterial stiffness measurements in patients with Behçet's disease (BD), using ultrasound (US) radiofrequency (RF) data technology.

Methods: We included 33 BD patients and 33 healthy controls in this study. The participants did not have any primary cardiovascular risk factors, such as diabetes mellitus, hypertension, hyperlipidemia and obesity. Read More

Front Immunol 2019 29;10:665. Epub 2019 Mar 29.

Department of Ophthalmology, The Second Hospital of Jilin University, Changchun, China.

Behcet's disease (BD) is a chronic systemic inflammatory vasculitis of unknown etiology characterized by recurrent episodes of oral aphthous ulcers, genital ulcers, skin lesions, ocular lesions, and other manifestations. Although the pathogenesis of BD is unclear, some studies have shown that immunological aberrations play an important role in the development and progression of BD. Infection-related trigger factors, including antigens and autoantigens, are believed to mediate the development of BD in patients with a genetic predisposition and subsequently activate the innate and adaptive immune systems, resulting in the production of numerous cytokines and chemokines to combat the infection-related factors. Read More

Korean J Ophthalmol 2019 Apr;33(2):167-172

Department of Ophthalmology, Inonu University School of Medicine, Malatya, Turkey.

Purpose: To evaluate contrast sensitivity in patients with Behçet's disease (BD) without ocular involvement.

Methods: The study group was composed of 47 BD patients (20 to 50 years of age) who did not have ocular involvement. The control group was composed of 47 normal volunteers who were similar to the study group in terms of age and gender. Read More

J Tehran Heart Cent 2018 Oct;13(4):180-182

Yozgat State Hospital, Yozgat, Turkey.

Behçet's Disease (BD) is a multisystemic vasculitis which usually affects optical, genital, and oral mucosae and often reoccurs intermittently. Chylothorax is a very rare complication of BD which usually causes thrombosis in the major venous system. A 27-year-old man with a 10-year history of BD referred to our cardiovascular surgery department with symptoms of serious aches in the left arm, edema, and apparent veins on the left anterior chest wall. Read More

Rev Med Interne 2019 Apr 4. Epub 2019 Apr 4.

Médecine interne et maladies systémiques, CHU Dijon Bourgogne, 21000 Dijon, France.

Background: Behçet's disease (BD) is a recurrent multisystemic disease responsible for occlusive vasculitis with arterial, venous and capillary involvement. The aim of this study was to determine the frequency and the features associated with the use of biotherapy in the management of patients followed in our department for BD.

Methods: This is a retrospective study of patients medical records followed for BD in a department of internal medicine from January 2005 to August 2018. Read More

Semin Arthritis Rheum 2019 Mar 9. Epub 2019 Mar 9.

DOIM-Rheum, MSC 10 5550, University of New Mexico Health Sciences Center, 5th FL ACC, Albuquerque, NM 87131, United States.

Objective: The goal of this study was to determine the characteristics of Behçet's disease (BD) in the American Southwest.

Material And Methods: This was a cross-sectional study of BD patients clinically encountered during a 2-year period. All subjects fulfilled the International Study Group criteria (ISG) or International Criteria for Behcet's Disease (ICBD). Read More

J Pediatr Hematol Oncol 2019 Apr 3. Epub 2019 Apr 3.

Division of Pediatric Hematology, Dr. Behçet Uz Children's Hospital, Izmir, Turkey.

Pyrimidine-5-nucleotidase (P5'N-1) deficiency is a rare nonspherocytic hemolytic anemia due to pyrimidine nucleotide deposition within erythrocytes. This rare erythrocyte disorder shows autosomal recessive inheritance with mutation of the pyrimidine-5'-nucleotidase gene, which is localized on 7p15-p14. Consanguinity of parents increases the probability of disease with novel mutations. Read More

J Nephrol 2019 Apr 4. Epub 2019 Apr 4.

Nephrology and Dialysis Unit, Pediatric Subspecialties Department, Institute for Scientific Research, Bambino Gesù Children's Hospital, Piazza S. Onofrio 4, 00165, Rome, Italy.

The number of children with acute kidney injury (AKI) requiring dialysis is increasing. To date, systematic analysis has been largely limited to critically ill children treated with continuous renal replacement therapy (CRRT). We conducted a survey among 35 European Pediatric Nephrology Centers to investigate dialysis practices in European children with AKI. Read More

Cell Mol Biol (Noisy-le-grand) 2019 Mar 31;65(3):32-40. Epub 2019 Mar 31.

Kent College, İzmir, Turkey.

Obesity and hyperandrogenemia are known to have adverse effects on both developing follicle and endometrium receptivity in polycystic ovarian syndrome (PCOS). Insulin resistance also contributes to this dilemma as a cause or a consequence and leads to worsening of the clinical picture. The difficulty in obtaining pregnancy despite the presence of a large number of oocyte has concentrated our attention on oocyte quality and development. Read More

Immunol Med 2018 Sep 30;41(3):120-128. Epub 2018 Nov 30.

a Department of Neurology and Clinical Neuroscience , Yamaguchi University Graduate School of Medicine , Ube , Japan.

The blood-brain barrier (BBB) is the brain-specific endothelial cell barrier that is important for maintaining brain homeostasis and preventing the entry of toxic substances. Pathological BBB dysfunction is a critical step of the disease process in several immuno-mediated neurological diseases, including multiple sclerosis (MS), neuromyelitis optica (NMO), neuropsychiatric systemic lupus erythematosus (NPSLE) and neuro-Behçet diseases. The pathological findings from patients with secondary progressive (SP) MS, NMO and NPSLE showed leaky BBB in the active lesions. Read More

World Allergy Organ J 2019 14;12(3):100019. Epub 2019 Mar 14.

Department of Dermatology and Allergy, Charité - Universitätsmedizin Berlin, Germany.

Background: Systemic autoinflammatory diseases (SAIDs) are rare debilitating disorders of which there is limited awareness and a significant delay in diagnosis. There is no uniform approach in the diagnosis and treatment of these disorders and the real life state of SAID patient care is poorly characterized. The aim of this study was to obtain data on the epidemiology, state of care and the perception of physicians who are involved in the care of SAID patients. Read More

Eur J Case Rep Intern Med 2019 1;6(3):001044. Epub 2019 Mar 1.

Pathology Department, Hospital da Luz, Lisbon, Portugal.

Behçet syndrome (BS) is a variable vessel vasculitis that has pleiotropic manifestations. A 43-year-old male with a previous diagnosis of Crohn's disease (CD) presented with deep venous thrombosis and bilateral superficial femoral artery aneurysms. A diagnosis of BS was made, and the patient was treated aggressively with immunosuppressive therapy and bilateral bypass surgery, attaining a favourable outcome. Read More

J Clin Lipidol 2019 Feb 21. Epub 2019 Feb 21.

Department of Cardiology, Hacettepe University Medical Faculty, Ankara, Turkey.

Background: Homozygous familial hypercholesterolemia (HoFH) is a rare, life-threatening inherited disease leading to early-onset atherosclerosis and associated morbidity. Because of its rarity, longitudinal data on the management of HoFH in the real world are lacking, particularly on the impact the condition has on quality of life (QoL), including the impact of the extracorporeal lipid removal procedure apheresis (LA).

Methods: The A-HIT1 study included 88 patients with HoFH aged ≥12 years receiving regular LA in 19 centers in Turkey. Read More

J Clin Res Pediatr Endocrinol 2019 Mar 25. Epub 2019 Mar 25.

Division of Pediatric Endocrinology, Dr Behcet Uz Children Training Hospital

Context: Idiopathic hypogonadotropic hypogonadism (IHH) is a rare disease caused by defects in the secretion of Gonadotropin releasing hormone (GnRH) or the action of GnRH on the pituitary gonadotrophes. is one of the genes, when mutated, cause IHH, and mutations of this gene are responsible for about 2-5% of patients with normosmic IHH (NIHH).

Objective: In this report, we aim to present three siblings who have NIHH due to a compound heterozygous mutation. Read More

J Clin Rheumatol 2019 Mar 20. Epub 2019 Mar 20.

Bristol Medical School, University of Bristol, Bristol, United Kingdom.

J Dent Sci 2018 Sep 7;13(3):256-262. Epub 2018 Jun 7.

Graduate Institute of Clinical Dentistry, School of Dentistry, National Taiwan University, Taipei, Taiwan.

Background/purpose: Our previous study found that 13 of 63 recurrent aphthous stomatitis (RAS)/Behcet's disease (BD) patients have thyroglobulin antibody (TGA) positivity and/or thyroid microsomal antibody (TMA) positivity (TGA/TMA positivity) but without gastric parietal cell antibody positivity. This study mainly assessed whether the serum TGA/TMA positivity was significantly associated with anemia, hematinic deficiencies, and hyperhomocysteinemia in TGA/TMA-positive RAS/BD patients.

Materials And Methods: The mean blood hemoglobin (Hb), iron, vitamin B12, folic acid, and homocysteine levels were measured and compared between 13 TGA/TMA-positive RAS/BD patients and 41 gastric and thyroid antibodies-negative RAS/BD patients (Abs־RAS/BD patients) or 126 healthy control subjects. Read More

Sci Rep 2019 Mar 20;9(1):4886. Epub 2019 Mar 20.

Children's Mercy Hospital, Kansas City, MO, USA.

While children approaching end-stage kidney disease (ESKD) are considered at risk of uremic anorexia and underweight they are also exposed to the global obesity epidemic. We sought to investigate the variation of nutritional status in children undergoing chronic peritoneal dialysis (CPD) around the globe. The distribution and course of body mass index (BMI) standard deviation score over time was examined prospectively in 1001 children and adolescents from 35 countries starting CPD who were followed in the International Pediatric PD Network (IPPN) Registry. Read More

Nan Fang Yi Ke Da Xue Xue Bao 2019 02;39(2):127-133

Department of Rheumatology, Huadong Hospital, Fudan University, Shanghai 200040, China.

Objective: Behcet's disease (BD) is an autoimmune disorder that causes most commonly mouth and genital ulcerations and erythema nodules of the skin and currently has limited options of therapeutic medicines. Metformin is recently reported to suppress immune reaction, and we hypothesized that metformin could be an option for treatment of BD.

Methods: Thirty patients with BD were enrolled in this perspective single-blinded, before-after study. Read More

Rheumatol Int 2019 Mar 19. Epub 2019 Mar 19.

Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Fatih, 34093, Istanbul, Turkey.

Arterial aneurysms are rare manifestations of Behçet Disease (BD) with high morbidity and mortality. This study aimed to investigate the clinical course of BD patients with abdominal aortic aneurysms (AAA). We retrospectively searched charts of BD patients, followed up between 1988 and 2011, to identify those with AAA with at least 6-month clinical and radiological follow-up data. Read More

Mult Scler Relat Disord 2019 Apr 19;29:154. Epub 2018 Dec 19.

Department of Neurology, Cliniques Universitaires St-Luc, Université catholique de Louvain (UCLouvain), 10 avenue Hippocrate, Brussels 1200, Belgium.

Mult Scler Relat Disord 2019 Apr 6;29:153. Epub 2019 Jan 6.

Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Hacettepe University, Gevher Nesibe Street, Ankara 06100, Turkey. Electronic address:

Intractable Rare Dis Res 2019 02;8(1):60-66

Venous Thromboembolism Unit, Department of Internal Medicine, Hospital General Universitario Gregorio Marañón, Madrid, Spain.

Behçet's Disease (BD) is a rare, chronic and recurrent inflammatory multisystemic condition of unknown origin that can affect any tissue. The vascular system is involved in 5-40% of cases of BD, including venous and arterial beds and it has a relapsing course. Budd-Chiari syndrome (BCS) is a rare complication of BD with a frequency of < 5% among patients with vascular involvement and is more frequent in men (89. Read More

Z Rheumatol 2019 Mar 14. Epub 2019 Mar 14.

Faculty of Behavioral, Management and Social Sciences, Department Psychology, Health and Technology, University of Twente, Enschede, The Netherlands.

Hughes-Stovin syndrome (HSS), characterized by the combination of multiple pulmonary artery aneurysms and deep vein thrombosis, is a rare and an under-recognized clinical entity with less than 40 published cases in English medical literature. Vascular venous thrombotic events, as occurring in the course of Behçet's disease (BD), are also described in HSS, e. g. Read More

Eur Heart J Cardiovasc Imaging 2019 Mar 14. Epub 2019 Mar 14.

Division of Cardiology, Department of Internal medicine, Soonchunhyang University Hospital, Soonchunhyang University College of Medicine, 170 Jomaru-ro, Bucheon 14584, South Korea.

N Engl J Med 2019 Mar;380(11):1062-1071

From the Departments of Ophthalmology, Medicine, and Cell Biology, Oregon Health and Science University, and Legacy Devers Eye Institute - both in Portland (J.T.R.); and the Department of Ophthalmology, Ophthalmic Consultants of Boston (L.M.R.), the Departments of Radiology (K.A.B.), Medicine (M.B.B.), and Pathology (M.P.H.), Massachusetts General Hospital, and the Departments of Radiology (K.A.B.), Medicine (M.B.B.), and Pathology (M.P.H.), Harvard Medical School - all in Boston.

J Neurol Neurosurg Psychiatry 2019 Mar 9. Epub 2019 Mar 9.

Neuroimmunology Clinic, Concord Hospital, University of Sydney, Sydney, NSW, Australia

The triad of central nervous system symptoms, visual disturbance and hearing impairment is an oft-encountered clinical scenario. A number of immune-mediated diseases should be considered among the differential diagnoses including: Susac syndrome, Cogan syndrome or Vogt-Koyanagi-Harada disease; demyelinating conditions such as multiple sclerosis or neuromyelitis optica spectrum disorder; systemic diseases such as systemic lupus erythematosus, Sjögren syndrome or Behcet disease and granulomatous diseases such as sarcoidosis. In this article, we coin the term 'BEE syndromes' to draw attention to the various immune-mediated diseases that affect the brain, eye and ear. Read More

J Fr Ophtalmol 2019 Apr 5;42(4):e133-e146. Epub 2019 Mar 5.

Service d'ophtalmologie, hôpital universitaire Fattouma Bourguiba, faculté de médecine, université de Monastir, 5019 Monastir, Tunisie. Electronic address:

Behçet's disease is a chronic multisystem disorder characterized by relapsing inflammation for which the underlying histopathology is an occlusive vasculitis. The disease has a high prevalence in the Mediterranean region, Far and Middle Eastern countries. It is clinically characterized by oral and genital ulcerations, ocular inflammatory involvement, skin lesions, vascular involvement and numerous other systemic manifestations. Read More

J Interferon Cytokine Res 2019 Mar 8. Epub 2019 Mar 8.

2 Medical Biochemistry and Molecular Biology Department, Faculty of Medicine, Cairo University, Cairo, Egypt.

Behçet's disease (BD) is a chronic multi-systemic inflammatory disease of uncertain pathogenesis and with no definitive diagnostic test. The aims of this study were to investigate serum levels of miR-181b in BD patients and to correlate this candidate biomarker with disease activity, cytokines, and adhesion molecules to identify new markers that can be used as a diagnostic tool for BD. Blood samples were collected from 96 participants who were classified according to their BD current activity form into 3 groups: healthy control, active BD, and inactive BD patients. Read More

Zhonghua Yan Ke Za Zhi 2019 Mar;55(3):203-207

Department of Neurology, Beijing Tongren Hospital, Capital Medical University, Beijing 100730, China.

To summarize the clinical manifestation, treatment and prognosis of anterior ischemic optic neuropathy(AION) which was the manifestation of optic neuropathy related with Behcet's disease (BD). Retrospective series of case studies. The clinical data of 6 cases (9 eyes) of AION associated with BD who were hospitalized at the neurology ward of Beijing Tongren Hospital from February to June in 2016 were collected, the clinical characteristics of these patients were summarized, visual acuity were compared by using Wingerchuk visual grade before and after treatment. Read More

Acta Myol 2018 09 1;37(3):210-220. Epub 2018 Sep 1.

University Hospital Cologne, Department of Pediatrics, Cologne, Germany.

The aim of this study is to analyze the epidemiology of the clinical and genetic features of childhood-onset limb-girdle muscular dystrophies (LGMD) in the Aegean part of Turkey. In total fifty-six pediatric cases with LGMD followed in four different pediatric neurology departments in the Aegean region of Turkey were evaluated. Among them, LGMD2C was the most common followed by LGMD2A, LGMD2D, and LGMD2F with equal frequencies. Read More

Eur J Intern Med 2019 Feb 25. Epub 2019 Feb 25.

University of Tunis El Manar, Faculty of Medicine of Tunis, LR99ES11 Tunis, Tunisia; Rabta Hospital, Laboratory of Biochemistry, Jebbari 1007, Tunis, Tunisia.. Electronic address:

Background: Behçet's disease (BD) is a multisystem inflammatory disease of unknown etiology. Beta-defensins are antimicrobial peptides involved in epithelial host defense. To explore whether beta-defensins might be involved in BD pathogenesis, we examined plasma human beta-defensin-1 (hBD-1) and DEFB1 -20G/A polymorphism in BD patients. Read More

Medicine (Baltimore) 2019 Mar;98(9):e14668

Department of Ophthalmology & Visual Science, Tokyo Medical and Dental University Graduate School of Medicine.

Noninfectious uveitis (NIU), which pathogenesis is often autoimmune nature, occurs as a symptom of systemic syndromes or only in the eye. The standard treatment of NIU is local, topical, and oral administration of corticosteroids (CS) in combination with immunomodulatory therapy (IMT). However, additional therapeutic strategies involving topical and systemic administration of CS or others to treat relapse or exacerbation of ocular inflammation in NIU which present as various ocular manifestations have not been established. Read More

Medicine (Baltimore) 2019 Mar;98(9):e14624

Department of Rheumatology and Immunology.

Rationale: Behçet's disease (BD) is a chronic immune-mediated inflammatory disorder involving multiple organ systems. In BD, intestinal ulcers can present as a refractory lesion capable of perforation, which makes the choice of treatment difficult.

Patient Concerns: A 34-year-old male who was diagnosed with intestinal BD and suffered with an ileocecal perforation. Read More

J Clin Immunol 2019 Feb 27;39(2):188-194. Epub 2019 Feb 27.

Department of Clinical Immunology, Children's Hospital of Fudan University, 399 Wanyuan Road, Shanghai, 201102, China.

Purpose: Tumor necrosis factor alpha-induced protein 3 (TNFAIP3, A20) is a negative regulator of the nuclear factor-κB (NF-κB) pathway. It has recently been recognized that TNFAIP3 deficiency leads to early onset of autoinflammatory and autoimmune syndrome resembling Behçet's disease. Here, we report a novel mutation in TNFAIP3 in a Chinese patient, who had Behçet-like phenotype and persistent Epstein-Barr virus (EBV) viremia. Read More

Ann Lab Med 2019 Jul;39(4):345-357

Departments of Rheumatology and Research and Development, Dudley Group NHS Foundation Trust (Teaching Trust of the University of Birmingham, UK), Russells Hall Hospital, Dudley, West Midlands, UK.

The platelet-to-lymphocyte ratio (PLR) has emerged as an informative marker revealing shifts in platelet and lymphocyte counts due to acute inflammatory and prothrombotic states. PLR has been extensively examined in neoplastic diseases accompanied by immune suppression and thrombosis, which can be predicted by combined blood cell counts and their ratios. Several large observational studies have demonstrated the value of shifts in PLR in evaluating the severity of systemic inflammation and predicting infections and other comorbidities, in inflammatory rheumatic diseases. Read More

Rheumatol Int 2019 Feb 25. Epub 2019 Feb 25.

SOTIRIA general Hospital, 11527, Athens, Greece.

Rheumatology has its roots in ancient Greece. Hippocrates and other prominent Greek (Hellenes) physicians in ancient times, Hellenistic, Roman, and Byzantine period were acute observers of disease course and of patients and were able to define many disorders. They wrote books on various aspects of medicine and these writings were the basis of medical practice and education in Europe and the Arabic world well into the seventeenth century. Read More

J Natl Med Assoc 2019 Feb 22. Epub 2019 Feb 22.

Department of Immunology and Rheumatology Division, Fudan University Huadong Hospital, #221 yan'an west Road, Shanghai 200040, PR China.

Background: To investigate the incidence, characteristics, and potential risk factors of anemia in patients with newly-diagnosed intestinal Behcet's disease (BD).

Methods: In this cross-sectional study, 106 newly-diagnosed intestinal BD patients were identified, and a gender-, age- and organ involvement-matched control group of 241 non-intestinal BD patients was established. Hemoglobin (Hb) levels below 120 g/L in women and 130 g/L in men were diagnosed as anemia; these were further classified as mild (Hb ≥ 90 g/L), moderate (60  g/L ≤ Hb < 90  g/L), and severe (Hb < 60 g/L) anemia for both genders. Read More

Rheumatol Int 2019 Feb 25. Epub 2019 Feb 25.

Department of Experimental and Clinical Medicine, University of Firenze, Florence, Italy.

Behçet's syndrome (BS) is a chronic (auto)-inflammatory disorder characterized by different clusters of symptoms, including mucocutaneous and ocular involvements. Interleukin-1 inhibitors anakinra (ANA), canakinumab (CAN), and gevokizumab (GEV) represent a promising therapeutic alternative in BS. To date, evidence on the use of ANA, CAN, and GEV is mainly based on small isolated studies or case series, and the real place of anti-IL1 agents in the treatment of BS is still unclear. Read More

Exp Ther Med 2019 Mar 24;17(3):1737-1741. Epub 2018 Dec 24.

Department of Rheumatology and Clinical Immunology, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong 510080, P.R. China.

Budd-Chiari syndrome (BCS) is a rare but severe venous form of Behcet's disease (BD) that is caused by the obstruction of the venous outflow tract that transports blood from hepatic veins into the inferior vena cava. In countries where BD is prevalent, including the Middle East and Far East, BCS awareness is important. In the present study, two cases of BCS are presented in two male Chinese patients with BD. Read More

J Vasc Surg Venous Lymphat Disord 2019 Feb 15. Epub 2019 Feb 15.

Academic Hospital, Istanbul, Turkey.

Objective: Lower extremity (LE) deep venous thrombosis (DVT) is the main feature of vascular involvement in Behçet disease (BD). We thought that vein wall thickness (VWT) could be a surrogate marker for venous inflammation and hence predict future vascular involvement. We assessed VWT in proximal LE veins in BD patients without DVT, BD patients with DVT, and healthy controls in a formal, masked protocol. Read More

J Med Vasc 2019 Feb 19;44(1):9-18. Epub 2018 Dec 19.

Vascular surgery department, faculty of medicine and pharmacy of Fez, Sidi Mohamed Ben Abdellah University, UHC Hassan II, Fez, Morocco.

Background: Behçet's disease (BD) is a multisystem chronic autoimmune inflammatory disorder that involves multiple organs. Arterial involvement in BD is rare. Aortic involvement is one of the most severe manifestations and is associated with a much higher mortality. Read More

Klin Monbl Augenheilkd 2019 Apr 14;236(4):487-491. Epub 2019 Feb 14.

Augenklinik, Universitätsspital Basel, Schweiz.

Acute posterior and panuveitis mostly affect younger patients and affect both eyes in more than half of cases. Because of the severe consequences in the clinical course, rapid and broad differential diagnosis are critical steps. Permanent loss of vision after a delay in starting therapy and the initiation of ineffective treatment are both serious risks. Read More

Intern Emerg Med 2019 Feb 11. Epub 2019 Feb 11.

Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, University of Istanbul, 81310, Istanbul, Turkey.

Behçet syndrome (BS) is a multi-systemic complex disorder with unknown etiology and a unique geographic distribution. It could not be possible to include it into specific classification schemes and it is certainly not a uniform disease. Several cluster and association studies revealed that it has been composed of multiple phenotypes ascribing the principal problem such as skin-mucosa, joint, eye, vascular, neurological and gastrointestinal involvement. Read More

Hum Immunol 2019 02 8. Epub 2019 Feb 8.

Rheumatology Institute of Lucania (IReL) and Rheumatology Department of Lucania, San Carlo Hospital of Potenza, via Potito Petrone, Potenza 85100, Italy; Electronic address:

The endoplasmic reticulum aminopeptidase protein 1 gene (ERAP1) is related to several human diseases, including Behçet syndrome (BS), a multisystemic disorder with unknown etiology. ERAP1 is involved in immune response and its role can be influenced by gene single nucleotide variations (SNVs). We genotyped the ERAP1 whole structure in 50 consecutive BS patients and 50 ethnically-matched healthy controls using both bioinformatics and molecular methodologies. Read More

Intern Emerg Med 2019 Feb 8. Epub 2019 Feb 8.

Department of Dermatology, Medicalpark Bahcelievler Private Hospital, Istanbul, Turkey.

Pathergy phenomenon is a non-specific tissue hyperreactivity reaction due to trauma and is a minor diagnostic criterion of Behcet's disease. In this study, 100 patients with a suspicion of Behcet's disease who were referred to Cerrahpasa Medical Faculty Dermatology department between 01.11. Read More

Ann Hum Genet 2019 May 7;83(3):177-186. Epub 2019 Feb 7.

Department of Clinical Immunology, School of Medicine, Ankara University, Ankara, Turkey.

Background: Behçet's disease (BD) is an inflammatory disorder characterized by oral aphthous lesions, uveitis, and genital ulcerations. The vitamin D receptor (VDR) has a crucial role in the pathogenesis of this disease because it mediates the functions of vitamin D in the immune system. Alterations of VDR expression related to polymorphic alleles of the VDR gene may play a pathogenic role in BD and BD's clinical presentations. Read More

N Engl J Med 2019 Feb;380(6):e7

Hospital Universitário Júlio Müller, Cuiaba, Brazil