Behcet&#39;s disease (BD) is a chronic disease which is characterized by recurrent oral apthous ulcerations, recurrent genital ulcerations, skin eruptions, ocular involvements and other various systemic manifestations as well as systemic vasculitis. Endocrine involvement in BD regarding various systems can be seen. Hypophysis is one of the best and dense vascularized organs of the body, thus it is likely that it can be affected by BD. Not only anterior hypophysis functions, but posterior hypophysis functions as well can be affected. As BD is a disease of autoimmune process, it may be possible that adrenal insufficiency or alterations in the cortisol levels could be expected. Another concern is whether or not there is insulin resistance in patients with BD. The avaliable data suggests that there is an increased susceptibility to insulin resistance in patients with BD.

Onur Ozhan

Kerem Sezer

Behcet&#39;s Disease and Endocrine System

Beh&#xE7;et&#39;s disease is a multisystemic inflammatory disease of unknown etiology which usually occurs as a trait of symptoms: aphthous stomatitis, genital ulcerations, and ocular disease. At the beginning of the disease the diagnosis is uncertain because of various clinical manifestations and a long period up to the full clinical picture manifestation. Since neither the laboratory data nor the histopathological signs are truly pathognomonic in Beh&#xE7;et&#39;s disease, the differential diagnosis depends on a careful evaluation of the medical history and meticulous physical examination to detect concomitant systemic manifestations. Sometimes, some laboratory test may help establish the diagnosis. Subspecialty referral to ophthalmology, rheumatology, neurology, and gastroenterology should be considered when indicated.

Aysin Kokturk

Clinical and Pathological Manifestations with Differential Diagnosis in Beh&#xE7;et&#39;s Disease

Beh&#xe7;et&#x27;s disease (BD) is a vasculitic disorder of relapsing acute inflammation characterized by recurrent oral ulcers, genital ulcers, uveitis, and skin lesions. The disease also affects other organs, including joints, the nervous system, blood vessels, and gastrointestinal (GI) system may also be involved and the lower GI tract is the mostly involved part, leading to severe morbidity. The frequency of GI involvement in BD varies among different ethnic groups. Although 50&#8211;60&#37; of Japanese patients have GI disease, these manifestations are rare in patients from Mediterranean countries. The gastrointestinal manifestations of BD usually appear 4.5&#8211;6 years after the onset of the oral ulcers. The intestinal lesions are usually resistant to medical treatment and recur after surgery. The elementary lesion is apthous ulcer. Deep, round or oval ulcers with a punched-out appearance tend to perforate easily, so that many patients require urgent operation.

Tuba Kara

Duygu D&#252;&#351;mez Apa

Pathologic Features of Beh&#xe7;et&#x27;s Disease in the Tubuler Gut

Beh&#231;et&#39;s disease (BD) is a multisystemic, relapsing inflammatory disorder with an obscure etiology and pathogenesis. Diagnosis depends on the clinician&#39;s ability to identify a group of nonspecific mucocutaneous lesions, which also manifest in a number of other diseases. In recent years, there has been an increase in the studies focusing on the histopathological aspects of Beh&#231;et&#39;s disease diagnostic mucocutaneous lesions. Their results emphasize the value of histopathology and direct immunofluorescence (DIF) in the differential diagnosis of Beh&#231;et&#39;s disease.

&#214;zg&#252;r G&#252;nd&#252;z

Histopathological Evaluation of Beh&#231;et&#39;s Disease and Identification of New Skin Lesions

Beh&#231;et&#39;s 
                  disease (BD) is a systemic inflammatory disorder 
                  characterized mainly by recurrent oral and 
                  genital ulcers and eye involvement. Although the 
                  pathogenesis remains poorly understood, a variety 
                  of studies have demonstrated that genetic 
                  predisposition is a major factor in disease 
                  susceptibility. Peculiar geographical 
                  distribution of BD along the ancient Silk Road 
                  has been regarded as evidence supporting 
                  genetic influence. The observed aggregation of 
                  BD in families of patients with BD is also 
                  supportive for a genetic component in its 
                  etiology. HLA-B51 (B510101 subtype) is the most 
                  strongly associated genetic marker for BD in 
                  countries on the Silk Road. In recent years, 
                  several genome-wide association studies and 
                  genetic polymorphism studies have also found new 
                  genetic associations with BD, which may have a 
                  supplementary role in disease susceptibility 
                  and/or severity. The author reviewed the HLA and 
                  non-HLA genetic association 
                  studies.

Tamer &#304;rfan Kaya

Genetics of Beh&#231;et&#39;s Disease

Beh&#231;et&#39;s 
                  disease (BD) is a chronic, relapsing, and 
                  debilitating systemic vasculitis of unknown 
                  aetiology with the clinical features of 
                  mucocutaneous lesions, ocular, vascular, 
                  articular, neurologic, gastrointestinal, 
                  urogenital, and pulmonary involvement. The 
                  disease is much more frequent along the ancient 
                  &#8220;Silk Route&#8221; extending from Eastern 
                  Asia to the Mediterranean basin, compared with 
                  Western countries. The disease usually starts 
                  around the third or fourth decade of life. Male 
                  sex and a younger age of onset are associated 
                  with more severe disease. Although the 
                  treatment has become much more effective in 
                  recent years, BD is still associated with severe 
                  morbidity and considerable mortality. The main 
                  aim of the treatment should be the prevention of 
                  irreversible organ damage. Therefore, close 
                  monitoring, early, and appropriate treatment is 
                  mandatory to reduce morbidity and mortality. The 
                  treatment is mainly based on the suppression of 
                  inflammatory attacks of the disease using 
                  immunomodulatory and immunosuppressive agents. 
                  In this paper, current state of knowledge 
                  regarding the therapeutic approaches is 
                  outlined. To provide a rational framework for 
                  selecting the appropriate therapy along the 
                  various treatment choices, a stepwise, 
                  symptom-based, evidence-based algorithmic 
                  approach was developed.

Erkan Alpsoy

New Evidence-Based Treatment Approach in Beh&#231;et&#39;s Disease

Beh&#231;et&#39;s disease (BD) is a multisystemic disease of unknown etiology characterized by chronic relapsing oral-genital ulcers and uveitis. Multiple systemic associations including articular, gastrointestinal, cardiopulmonary, neurologic, and vascular involvement are also observed in BD. Although the etiopathogenesis of the disease remains unknown, increased neutrophil functions such as chemotaxis, phagocytosis, and excessive production of reactive oxygen species (ROS), including superoxide anion, which may be responsible for oxidative tissue damage seen in BD, and also immunological alterations, T lymphocyte abnormalities in both subpopulation and function have been considered to be correlated with the etiopathogenesis of BD. There is some clinical evidence suggesting that emotional stress and hormonal alterations can influence the course and disease activity of BD.

&#220;mit T&#252;rsen

Pathophysiology of the Beh&#231;et&#39;s Disease

Historical Background. The ISG criteria for Behcet&#39;s, created in 1990, have excellent specificity, but lack sensitivity. The International Criteria for Behcet&#39;s Disease (ICBD) was created in 2006, as replacement to ISG. The aim of this study was to compare their performance. ISG and ICBD Criteria. For ISG oral aphthosis is mandatory. The presence of any two of the following (genital aphthosis, skin lesions, eye lesions, and positive pathergy test) will diagnose/classify the patient as BD. For ICBD, vascular lesions were added, while oral aphthosis is no more mandatory. Getting 3 or more points diagnose/classify the patient as BD (genital aphthosis 2 points, eye lesions 2 points, and the remaining each one point). Performance and Comparison of ISG and ICBD. Their sensitivity, specificity, and accuracy (percent agreement), were tested in three independent cohort of patients from Far-East (China), Middle-East (Iran), and Europe (Germany). The sensitivity for ISG was respectively 65.4&#37;, 78.1&#37;, 83.7&#37; and for ICBD 87&#37;, 98.2&#37;, and 96.5&#37;. The specificity for ISG was 99.2&#37;, 98.8&#37;, 89.5&#37; and for ICBD 94.1&#37;, 95.6&#37;, and 73.7&#37;. The accuracy for ISG was 74.2&#37;, 85.5&#37;, 85.5&#37; and for ICBD 88.9&#37;, 97.3&#37;, and 89.5&#37;. Conclusion. ICBD has better sensitivity, and accuracy than ISG.

Fereydoun Davatchi

Diagnosis/Classification Criteria for Behcet&#39;s Disease

Behcet&#39;s disease is a multisystem disease characterized by recurrent oral and genital ulcers, relapsing uveitis, mucocutaneous, articular, gastrointestinal, neurologic, and vascular manifestations. Rheumatologic manifestations may also occur in Behcet&#39;s disease, and arthritis and arthralgia are the most common musculoskeletal findings followed by enthesopathy, avascular necrosis, myalgia, and myositis. Although the main pathology of Behcet&#39;s disease has been known to be the underlying vasculitis, the etiology and exact pathogenesis of the disease are still unclear. Musculoskeletal findings of Behcet&#39;s disease, the relationship between Behcet&#39;s disease and spondyloarthropathy disease complex, and the status of bone metabolism in patients with Behcet&#39;s disease were discussed in this paper.

Ali Bicer

Musculoskeletal Findings in Behcet&#39;s Disease

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Table of Contents

Table of Contents: 2012

Behcet's Disease and Endocrine System

Clinical and Pathological Manifestations with Differential Diagnosis in Behçet's Disease

Pathologic Features of Behçet's Disease in the Tubuler Gut

Histopathological Evaluation of Behçet's Disease and Identification of New Skin Lesions

Genetics of Behçet's Disease

New Evidence-Based Treatment Approach in Behçet's Disease

Pathophysiology of the Behçet's Disease

Diagnosis/Classification Criteria for Behcet's Disease

Musculoskeletal Findings in Behcet's Disease