Behcet's disease (BD) is a multi-systemic inflammatory disorder of an unknown etiology and shows a chronic recurrent clinical course. When the disease involves the alimentary tract, it is called intestinal BD because of its clinical importance. Intestinal BD is more frequently reported in East Asian countries than in Western or Middle Eastern countries. While any part of the gastrointestinal tract can be involved, the most common location of intestinal BD is the ileocecal area. A few, large, deep ulcerations with discrete border are characteristic endoscopic findings of intestinal BD. Currently, there is no single gold standard test or pathognomonic finding of intestinal BD. However, recently developed novel diagnostic criteria and a disease activity index have helped in assessing intestinal BD. As intestinal BD shares a lot of characteristics with inflammatory bowel disease, including genetic background, clinical manifestations, and therapeutic strategies, distinguishing between the two diseases in clinical practice is quite difficult. However, biologic agents such as anti-tumor necrosis factor alpha antibody shows a considerable efficacy similar to inflammatory bowel disease cases. It is important to distinguish and treat those two disease entities separately from the standpoint of precise medicine. Clinicians should require comprehensive knowledge regarding the similarities and differences between intestinal BD and inflammatory bowel disease for making an accurate clinical decision.
Behcet Syndrome/*diagnosis/therapy ; Diagnosis, Differential ; Gastrointestinal Diseases/*diagnosis/therapy ; Humans ; Inflammatory Bowel Diseases ; Male ; Middle Aged ; Systemic Vasculitis/*diagnosis/etiology ; Treatment Outcome ; Tumor Necrosis Factor-alpha/therapeutic use

No abstract available.
Aneurysm, Dissecting/diagnosis/*etiology/physiopathology/therapy ; Anticoagulants/therapeutic use ; Aortic Aneurysm/diagnosis/*etiology/physiopathology/therapy ; Behcet Syndrome/*complications/diagnosis/drug therapy ; Cerebral Infarction/diagnosis/etiology ; Diffusion Magnetic Resonance Imaging ; Echocardiography, Doppler, Color ; Electrocardiography ; Hemodynamics ; Humans ; Immunosuppressive Agents/therapeutic use ; Male ; Middle Aged ; *Sinus of Valsalva/physiopathology/ultrasonography ; Thrombosis/diagnosis/drug therapy/*etiology/physiopathology ; Ventricular Septal Rupture/diagnosis/*etiology/physiopathology/therapy

Behcet's disease (BD) involves multisystem vasculitis of unknown origin. Ocular manifestations of BD mostly include bilateral panuveitis and retinal vasculitis, which are very challenging to treat. Interferon alfa-2a (IFN) has been recently introduced for treating refractory Behcet uveitis, mainly in Germany and Turkey. Nonetheless, there is so far no consensus about the ideal treatment regimen of IFN for Behcet uveitis. We report our experience of IFN treatment in five Korean BD patients with refractory uveitis. All patients complained of oral ulcers; one patient had a positive pathergy test and 2 showed the presence of HLA-B51. Immunosuppressive agents used prior to IFN treatment included cyclosporine and methotrexate. The IFN treatment was commenced with a dose of 6-9 MIU/day for 7 days, adjusted according to individual ocular manifestations, tapered down to 3 MIU three times in a week, and then discontinued. All patients showed positive response to IFN treatment; 50% of them showed complete response without additional major ocular inflammation during the follow-up period. Other BD symptoms also improved after IFN treatment in most cases. After treatment, the relapse rate and the required dose of oral corticosteroid were decreased in most cases, showing a significant steroid-sparing effect. However, the visual acuity was not improved in most cases due to irreversible macular sequelae. Despite the small sample size of this study, we suggest that, in Korean patients, IFN is an effective treatment modality for BD uveitis as was observed in German and Turkish patients.
Adult ; Behcet Syndrome/complications/diagnosis/*drug therapy ; Chronic Disease ; Cyclosporine/therapeutic use ; Female ; Humans ; Immunosuppressive Agents/administration & dosage ; Interferon-alpha/*therapeutic use ; Male ; Recombinant Proteins/therapeutic use ; Recurrence ; Remission Induction ; Treatment Outcome ; Turkey ; Uveitis/diagnosis/*drug therapy/etiology ; Visual Acuity

No abstract available.
Behcet Syndrome/*complications/diagnosis/drug therapy ; Drug Therapy, Combination ; Hemianopsia/diagnosis/*etiology/physiopathology ; Humans ; Immunosuppressive Agents/administration & dosage ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Pulse Therapy, Drug ; Steroids/administration & dosage ; Treatment Outcome ; Visual Field Tests ; Visual Fields

Behcet's disease (BD) is a multisystem inflammatory disorder that presents as recurrent oral and genital ulcers in conjunction with other dermatological and ocular manifestations. The prevalence of BD is higher in Middle and East Asia than in Western countries. Intestinal BD is a specific subtype of BD, characterized by intestinal ulcers and associated gastrointestinal symptoms. Similar to inflammatory bowel disease, intestinal BD exhibits a fluctuating disease course with repeated episodes of relapse and remission that necessitate adequate maintenance therapy after achievement of clinical remission. Medical treatment of intestinal BD is largely empirical since well-controlled studies have been difficult to perform due to the heterogeneity and rarity of the disease. To date, 5-aminosalicylic acid, systemic corticosteroids, and immunosuppressants have been used anecdotally to treat intestinal BD. The clinical course of intestinal BD shows considerable variability, and the exact point at which more potent agents such as immunosuppressants should be used has not yet been elucidated. Given the difficulty in predicting which patients will experience complicated disease courses and the fact that these drugs are related with certain risk resulting from immunosuppression, proper identification of prognostic factors in intestinal BD may allow physicians to implement tailored medical therapy and individualized patient monitoring based on risk stratification. In this review, the impact of baseline characteristics on the long-term course of intestinal BD, prognostic factors during various medical therapies, and outcome predictors related to surgery will be discussed.
Adrenal Cortex Hormones/adverse effects/therapeutic use ; Adult ; Age Factors ; Behcet Syndrome/*diagnosis/pathology/therapy ; Female ; Humans ; Immunosuppressive Agents/adverse effects/therapeutic use ; Immunotherapy ; Intestinal Diseases/*diagnosis/pathology/therapy ; Male ; Prognosis ; Sex Factors

INTRODUCTIONThough oral aphthosis is common, it has a significant impact on the quality of life in the patients. It is the most common oral ulcerative condition encountered in clinical practice. This study describes the characteristics and patterns of oral aphthosis seen at a tertiary dermatological centre in Singapore, with emphasis in evaluating the management gaps and in identifying underlying systemic diseases and nutritional deficiencies.

MATERIALS AND METHODSThis is a retrospective review of medical records over a 10-year period between June 2000 and June 2010. Two hundred and thirteen patients were identified using the search terms 'oral ulcers', 'aphthous ulcers', 'oral aphthosis', and 'Behcet's disease'. Patients with Behcet's disease without oral ulcers and other diagnoses such as pemphigus vulgaris, lichen planus and herpes simplex were excluded. The remaining patients were evaluated with regard to demographic characteristics, characteristics of oral ulcers, associated connective tissue disorders and nutritional deficiencies, diagnostic tests results, treatment response as well as follow-up duration.

RESULTSOne hundred and seventy-fi ve patients were included in this study. One hundred and one patients had recurrent oral aphthosis, with 77 having simple aphthosis and 24 having complex aphthosis. Fourteen patients (8%) fulfilled the International Study Criteria (ISG) for Behcet's disease, of which, 85.71% had complex aphthosis. The therapeutic ladder for such patients ranged from topical steroids and colchicine through to oral corticosteroids and/or dapsone therapy.

CONCLUSIONRecurrent oral aphthosis is a niche condition in which dermatologists are well-poised to manage. This study demonstrates that a more definitive management and therapeutic algorithm for oral aphthosis are needed for better management patients in the future. In particular, complex aphthosis needs to be monitored for progression onto Behcet's disease.

Adolescent ; Adrenal Cortex Hormones ; therapeutic use ; Adult ; Aged ; Aged, 80 and over ; Anti-Infective Agents ; therapeutic use ; Anti-Inflammatory Agents ; therapeutic use ; Behcet Syndrome ; complications ; Child ; Child, Preschool ; Colchicine ; therapeutic use ; Dapsone ; therapeutic use ; Drug Therapy, Combination ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Recurrence ; Retrospective Studies ; Stomatitis, Aphthous ; diagnosis ; drug therapy ; etiology ; Treatment Outcome ; Tubulin Modulators ; therapeutic use ; Young Adult

We report a 39-yr-old man with neuro-Behcet's disease (NBD) in remission who developed left-sided ataxia with a sensory deficit about 10 days after tooth extraction. Several years ago, he experienced a similar episode of relapse after tooth extraction. Brain magnetic resonance imaging showed a newly developed right thalamic lesion. In cerebrospinal fluid, lymphocyte-dominant pleocytosis and mild elevation of IgG were found. Immunologic factors may be important in the pathogenesis of NBD because of the time delay between tooth extraction and relapse. Careful observation and prevention are needed before dental procedures in patients with NBD.
Adult ; Behcet Syndrome/*diagnosis/drug therapy ; Brain/radionuclide imaging ; Humans ; Immunoglobulin G/analysis ; Leukocytosis/cerebrospinal fluid/diagnosis ; Magnetic Resonance Imaging ; Male ; Methylprednisolone/therapeutic use ; Neuroprotective Agents/therapeutic use ; Recurrence ; *Tooth Extraction

PURPOSE: The Behcet's Disease Quality of Life (BD-QoL) is a BD-specific measure developed in the UK. The aim of this study was to adapt the BD-QoL for use in Korea. PATIENTS AND METHODS: The translation was based on the guidelines for cross-cultural adaptation. A total of 201 Korean patients with BD participated in this study. To evaluate the psychometric properties, internal consistency and test-retest reliability were used. Factor analysis was performed to examine the construct validity. To provide further evidence for validity, the correlation of BD-QoL with the Clinical Activity Form for Korean Patients with BD (BDCAF-K) and the Center for Epidemiologic Studies-Depression (CES-D) scales was assessed. RESULTS: The Korean version had high internal consistency (Cronbach's alpha, 0.93) and test-retest reliability (r = 0.835). Factor analysis of the questionnaire revealed one interpretable factor as a general health-related quality of life factor. The Korean version significantly correlated with scores of CES-D (r = 0.749, p = 0.000), self-rating scale of well-being over the past 28 days (r = 0.446, p = 0.000), and BDCAF-K score (r = 0.502, p = 0.000). CONCLUSION: Adaptation of the BD-QoL for use in Korea was successful. Together with the BDCAF-K, it may be a valuable tool for assessing the influence of interventions in BD patients and outcome in clinical trials.
Adolescent ; Adult ; Aged ; Behcet Syndrome/diagnosis/*psychology/therapy ; Child ; Cultural Characteristics ; Female ; Humans ; Korea ; Language ; Male ; Middle Aged ; *Quality of Life ; Questionnaires/*standards

Behcet's disease is a multi-systemic vasculitis and characterized by systemic organ involvement. Although the gastrointestinal and systemic features of Behcet's disease and inflammatory bowel disease overlap to a considerable extent, they are generally viewed as two distinct diseases. A 39-yr-old female was diagnosed as having Behcet's disease. She was admitted to our hospital because of oral and genital ulcer, lower abdominal pain, and frequent diarrhea. Colonosopy showed diffuse involvement of multiple longitudinal ulcers with inflammatory pseudopolyps with a cobblestone appearance and ano-rectal fistula was suspected. These findings are extremely rare in Behcet's disease. However, there were no granulomas, the hallmark of Crohn's colitis. Microscopically, perivasculitis and multiple lymph follicles compatible with Behcet's disease were seen. Although being rarely encountered, multiple longitudinal ulcers, cobblestone appearance, and ano-rectal fistula can develop in Behcet's disease, as in Crohn's colitis. Therefore, Behcet's disease and Crohn's disease may be closely related and part of a spectrum of disease.
Adult ; Behcet Syndrome/diagnosis/*therapy ; Colitis ; Colonoscopy ; Crohn Disease/diagnosis/*therapy ; Female ; Humans ; Inflammation ; Inflammatory Bowel Diseases/pathology ; Intestinal Diseases/diagnosis/*therapy ; Perineum/pathology ; Ulcer ; Vulva/pathology

Approximately 50 % of patients with scleritis are known to be associated with systemic connective tissue diseases or vasculitic diseases such as rheumatoid arthritis (RA), Wegener's granulomatosis, relapsing polychondritis, and systemic lupus erythematosus. The patients with scleritis in association with Behcet's disease (BD) have been rarely described in the literature. We report a 46-year-old female patient with BD who developed the nodular scleritis.
Anti-Inflammatory Agents, Non-Steroidal/therapeutic use ; Behcet's Syndrome/drug therapy ; Behcet's Syndrome/diagnosis* ; Behcet's Syndrome/complications* ; Case Report ; Cyclophosphamide/therapeutic use ; Drug Therapy, Combination ; Female ; Follow-Up Studies ; Human ; Middle Age ; Prednisolone/therapeutic use ; Scleritis/drug therapy ; Scleritis/diagnosis* ; Scleritis/complications*