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

BACKGROUND/AIMS: Anemia in patients with inflammatory bowel disease significantly affects the quality of life. The aim of this study was to investigate the frequency of and risk factors for anemia and to describe the management of anemia in patients with intestinal Behcet's disease (BD) in actual clinical practice. METHODS: We included 64 patients with intestinal BD who visited the outpatient clinic of a tertiary referral center in June 2011 and had available laboratory data for the subsequent 6 months. RESULTS: Anemia was detected in 26 patients (40.6%). After 6 months, anemia was still present in 14 of these patients (53.8%). The cause of anemia was investigated in eight patients (30.8%), and oral iron supplementation was prescribed to four patients (15.4%). Of these four patients, two (50%) recovered completely within 6 months. Anemia was associated with a high Disease Activity Index for Intestinal Behcet's Disease (DAIBD, p=0.024), erythrocyte sedimentation rate (p=0.003), and C-reactive protein (p=0.049) in univariate analysis. In multivariate analysis, the factor predictive for anemia in patients with intestinal BD was a higher DAIBD (> or =40; odds ratio, 4.08; 95% confidence interval, 1.21 to 13.71; p=0.023). CONCLUSIONS: Although anemia is common in intestinal BD patients, its clinical importance is overlooked in daily practice. Moderate to severe disease activity is predictive of anemia.
Adult ; Anemia/drug therapy/epidemiology/*etiology ; Behcet Syndrome/blood/*complications/pathology ; Blood Sedimentation ; C-Reactive Protein/analysis ; Dietary Supplements ; Disease Management ; Female ; Humans ; Intestinal Diseases/blood/*complications/pathology ; Iron/therapeutic use ; Male ; Middle Aged ; Multivariate Analysis ; Odds Ratio ; Predictive Value of Tests ; Risk Factors ; Severity of Illness Index ; Trace Elements/therapeutic use

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

PURPOSE: Behcet's disease (BD) is rarely reported to be associated with malignancies in the literature. However, the frequency of cancer in BD patients remains unknown. This study evaluated cancer morbidity in BD patients compared with that in the general population of Korea. MATERIALS AND METHODS: A retrospective chart review was performed on 506 patients visiting our hospital from 1994 to 2011 for BD. We analyzed the standardized morbidity rate (SMR), which is the ratio of observed to expected malignancies. Furthermore, we reviewed cases of solid cancer in BD patients in the literature. RESULTS: Of the 506 patients with BD, 11 (2.17%) developed cancer. We found a variety of solid cancers without predominance and no hematologic malignancies. The total number of cancers observed was less than expected, which was determined from the statistical data of the National Cancer Information Center of Korea, with an SMR of 0.023 (95% confidence interval, 0.012-0.039). CONCLUSION: BD may be associated with a lower cancer-related morbidity compared with the general population of Korea.
Adult ; Behcet Syndrome/*complications/drug therapy/epidemiology ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Neoplasms/*epidemiology/etiology/surgery ; Republic of Korea/epidemiology ; Retrospective Studies

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

BACKGROUND/AIMS: This study was to evaluate the frequency and the course of the adverse effects of AZA/6-MP in Korean patients with inflammatory bowel disease (IBD). METHODS: Medical records of the patients with IBD treated with AZA/6-MP at Severance hospital from June 1996 to September 2006 were retrospectively analyzed. RESULTS: A total of 133 patients were studied. Male to female ratio was 1.3:1. The mean age was 31.7+/-10.9 year. Adverse effects included leukopenia occurred in 75 cases (56.4%), nausea/vomiting in 32 cases (24.1%), arthralgia in 6 cases (4.5%), hepatitis in 6 cases (4.5%), skin rash in 4 cases (3.0%), herpes zoster in 3 cases (2.3%), and headache in 1 case (0.8%). Most of leucopenia (58.7%) developed within 3 months after maximal tolerated dose of AZA/6-MP and nausea/vomiting frequently occurred within 3 months after start of AZA/6-MP treatment. Thirty-eight patients (28.6%) required the discontinuation of medication due to adverse effects. CONCLUSIONS: Leukopenia was the most common adverse effect of AZA/6-MP treatment. Leukopenia and nausea/vomiting developed frequently in the early period of treatment of AZA/6-MP in patients with IBD. AZA/6-MP should be used cautiously to scrutinize bone marrow suppression.
6-Mercaptopurine/*adverse effects/therapeutic use ; Adolescent ; Adult ; Azathioprine/*adverse effects/therapeutic use ; Behcet Syndrome/drug therapy/etiology ; Cohort Studies ; Drug Therapy, Combination ; Female ; Humans ; Immunosuppressive Agents/*adverse effects/therapeutic use ; Inflammatory Bowel Diseases/*drug therapy/etiology ; Leukopenia/chemically induced ; Male ; Middle Aged ; Retrospective Studies ; Time Factors

Behcet's disease (BD) is a chronic relapsing multisystem disease characterized by oral ulceration, genital ulceration and ocular lesions. Gastrointestinal involvement is rare, often difficult to treat and associated with a high mortality rate. We treated a 47-year-old Korean man with BD who had a recurrent intestinal ulcer with tumor necrosis factor alpha antibody (infliximab); he initially underwent right hemicolectomy due to uncontrolled intestinal bleeding. For patients with intestinal BD who fail to respond to conventional treatment, infliximab may be a safe and effective new therapeutic option.
Tumor Necrosis Factor-alpha/*therapeutic use ; *Treatment Outcome ; Remission Induction ; Middle Aged ; Male ; Humans ; Gastrointestinal Diseases/*drug therapy/etiology/surgery ; Gastrointestinal Agents/*therapeutic use ; Disease Progression ; Colectomy ; Behcet Syndrome/*drug therapy/physiopathology/surgery ; Antibodies, Monoclonal/*therapeutic use

Intestinal Behcet's disease (BD) refers to colonic ulcerative lesions documented by objective measures in patients with BD. Although the causes of intestinal BD are unknown, genetic, environmental, and immunological factors have been suggested. Intestinal BD is common in BD patients from Far East, while it is uncommon in those from the Middle East. The reasons for such peculiar geographic distribution in intestinal BD are unknown, but may provide clues for the elucidation of putative etiological agents or genetic factors that might be associated with intestinal BD. Although the treatment of Crohn's disease has improved significantly during past decade, the treatment of intestinal BD is still problematic. Corticosteroids, sulfasalazine, immunomodulators, and colchicines have been used to treat intestinal BD with varying degree of success. Thalidomide and its analogues also appear to be applicable. Monoclonal antibodies to TNF-alpha have recently been focused as a novel therapeutic option for patients with intestinal BD.
Adrenal Cortex Hormones/therapeutic use ; Antibodies, Monoclonal/therapeutic use ; Behcet Syndrome/*drug therapy/*etiology ; Colchicine/therapeutic use ; Colitis, Ulcerative/*drug therapy/*etiology ; Crohn Disease/etiology ; Humans ; Immunologic Factors/therapeutic use ; Prognosis ; Sulfasalazine/therapeutic use ; Thalidomide/therapeutic use ; Tumor Necrosis Factor-alpha/immunology

OBJECTIVES: To assess the clinical features, pathologic findings, postoperative results and the effects of immunosuppressive therapy in patients with Beh et's disease (BD). METHODS: We reviewed the postoperative course of the 9 BD patients who underwent a total of 17 aortic valve replacement procedures with prosthetic valves. RESULTS: Histological examination of the aortic valve commonly revealed diffuse myxoid degeneration (75 percent). Of 17 valve replacement surgeries, 13 surgeries resulted in complications, such as detachment of the prosthetic valve with perivalvular leakage and dehiscence of the sternotomy wound, within an average of 5 months (range from 1 month to 14 months). The rate of prosthetic valve detachment was 76 percent (13 of 17 surgeries). Four of the 9 patients (44 percent) who underwent aortic valve replacement procedures died of heart failure or infection associated with the detachment of the prosthetic valve, and perivalvular leakage within an average of 9 months. Aortic insufficiency associated with dehiscence of the prosthetic valve developed in 11 of 12 surgical cases (92 percent) with a mechanical valve and 2 of 5 surgical cases (40 percent) with tissue valves. Thirteen of 15 surgeries (87 percent) which were not given postoperative immunosuppressive therapy developed complications, while none of 2 surgeries that used postoperative immunosuppressive therapy with prednisolone (1 mg/kg/day) and azathioprine (100 mg/day) had these complications. CONCLUSION: The rates of prosthetic valve detachment in BD involving aortic valve were higher than those of other diseases. Aortic valve involvement was also one of the poor prognostic factors in BD. Intensive postoperative immunosuppressive therapy and surgical methods may be important factors for postoperative results.
Adult ; Aortic Valve/pathology ; Aortic Valve Insufficiency/*etiology/pathology ; Behcet Syndrome/*complications/drug therapy/pathology ; Female ; Heart Valve Diseases/*complications/pathology/surgery ; Heart Valve Prosthesis Implantation/*mortality ; Human ; Immunosuppression ; Male ; Postoperative Complications ; Prosthesis Failure ; Survival Analysis