Loss of vision in Behcet's disease is one of the most common, as well as one of the most serious, of its varied manifestations. Total blindness may often be the eventual outcome. The underlying problem in the eye, as well as in other organ systems, is an occlusive vasculitis. Although the most common ocular symptom is that of iridocyclitis, the presence of retinovascular lesions, especially necrotizing retinitis, is well known but often obscured by the severity of the anterior reaction, which precludes a good view of the retina. While Behcet's disease is characterized by spontaneous remissions and relapses, no external factors have been found to explain the length of remissions. Blindness, which occurs in up to 25% of patients, is one of the major causes of permanent disability.
Behcet's Syndrome/therapy ; Behcet's Syndrome/pathology* ; Eye/pathology* ; Human ; Prognosis

Behcet's disease is particularly prevalent in "Silk Route" populations, but it has a global distribution. The diagnosis of the disease is based on clinical criteria as there is as yet no pathognomonic test, and mucocutaneous lesions, which figure prominently in the presentation and diagnosis, may be considered the diagnostic hallmarks. Among the internationally accepted criteria, painful oral and genital ulcers, cutaneous vasculitic lesions and reactivity of the skin to needle prick or injection (the pathergy reaction) are considered hallmarks of Behcet's disease, and often precede other manifestations. Their recognition may permit earlier diagnosis and treatment, with salutary results. This paper describes the various lesions that constitute the syndrome and focuses on those that may be considered characteristic.
Behcet Syndrome/drug therapy/*pathology ; Female ; Humans ; Male ; Oral Ulcer/drug therapy/pathology ; Skin Ulcer/drug therapy/pathology ; Thrombophlebitis/drug therapy/pathology

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

We were able to identify 7 patients who died of Behcet's disease, among 2,200 patients registered at the Behcet's Disease Specialty Clinic of Severance Hospital from November 1983 to October 1992. Six were male and one female. Age of death was 31 to 55 years with the mean age of 39.1. The age of onset was 24 to 54 years with the mean age of 32.6. The mean duration of illness was 6.5 years. The most frequent initial symptom was oral ulcer, with the most common type being the incomplete type in Shimizu's classification. Positive pathergy tests were found in all the 5 patients in whom the test was performed out of 7 patients. Various modalities of treatments such as oral steroid, colchicine, azathioprine, colectomy, and pacemaker insertion were attempted without outstanding therapeutic effects. The causes of death were gastrointestinal bleeding, bowel perforation, superior and inferior vena cava syndrome, aortic regurgitation, cerebrovascular disease, sepsis, and lung abscess.
Adult ; *Behcet Syndrome/pathology/therapy ; Cause of Death ; Fatal Outcome ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged

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

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

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

Rheumatoid arthritis (RA) is a multifactorial autoimmune disease whose etiopathogenesis is not well understood. Although soluble (s) forms of 4-1BB (s4-1BB) and 4-1BB legand (s4-1BBL) have been detected in the sera of RA patients, their significance is not known. We compared the serum levels of s4-1BB and s4-1BBL in RA patients with those in systemic lupus erythematosus (SLE) and Behcet's disease (BD) patients. Serum levels of s4-1BB and s4-1BBL were significantly higher in RA patients compared with healthy controls, SLE or BD patients, and the abundance was correlated with disease severity in patients with RA. The serum levels of s4-1BB in RA patients were inversely corroborated with 4-1BB expression levels on activated T lymphocytes. In addition, there was a correlation between serum levels of s4-1BB and s4-1BBL. The augmented secretion of s4-1BB and s4-1BBL levels into the serum may reflect the clinical symptoms of RA and levels of s4-1BB and s4-1BBL in sera at the time of diagnosis may be indicative of the severity and outcome of RA.
Adult ; Aged ; Antigens, CD/metabolism ; Arthritis, Rheumatoid/*blood/drug therapy/immunology/*pathology ; Behcet Syndrome/blood/immunology ; Comparative Study ; Female ; Humans ; Immunosuppressive Agents/metabolism/therapeutic use ; Leukocytes, Mononuclear/metabolism ; Lupus Erythematosus, Systemic/blood/immunology ; Male ; Middle Aged ; Random Allocation ; Receptors, Nerve Growth Factor/*blood ; Receptors, Tumor Necrosis Factor/*blood ; Research Support, Non-U.S. Gov't ; Research Support, U.S. Gov't, P.H.S. ; Severity of Illness Index ; Statistics ; Tumor Necrosis Factor-alpha/*metabolism