Intestinal Behcet’s : A Rare Presentation of Behcet’s Disease

We herein present a case of 48 years old female patient presented with fever, bloody diarrhea followed by palpable purpuric rash over the body along with recurrent oral and genital ulceration. These were associated with history of symmetric polyarthralgia. On examination moderate anemia, signs of anterior uveitis were found. In blood parameters thrombocytopenia along with elevated Erythrocyte Sedimentation Rate, C-Reactive Protein were noted. On further investigations the serological tests were found to be negative for Dengue, Chikungunya, HIV, HBV, HCV. Complement C3 found to be low. Colonoscopic biopsy is diagnostic of Indeterminate Crohn’s Disease with IgA, G, M, C3, Fibrinogen immunostaining in skin biopsy. ANA, P-ANCA, C-ANCA were found to be nonreactive for the patient. All of the above mentioned points were pointing towards Behcet’s disease. For confirmation, Anti Saccharomyces Cerevisae Antibody was found to be positive . Skin pathergy test was positive. So, we diagnosed this case as behcet’s disease.

Chinese Consensus on Diagnosis and Treatment of Intestinal Behçet’s Syndrome / 胃肠病学

Behçet’s syndrome is a kind of chronic systemic vasculitis with involvement of multiple organs. Intestinal involvement of Behçet’s syndrome is presently named as intestinal Behçet’s syndrome (disease). Recently, there is considering another kind of disease type with only typical intestinal ulcers. Since it is difficult to differentiate intestinal Behçet’s syndrome from Crohn’s disease, intestinal tuberculosis, intestinal lymphoma, as well as intestinal manifestations of many other autoimmune diseases, and there is limited evidence for the therapy of intestinal Behçet’s syndrome, proposing diagnosis and treatment recommendations for intestinal Behçet’s syndrome through evidence-based judgment will be of great significance for clinical practice.

Anti-Tumor Necrosis Factor Therapy in Intestinal Behçet's Disease

Intestinal Behçet's disease is a rare, immune-mediated chronic intestinal inflammatory disease; therefore, clinical trials to optimize the management and treatment of patients are scarce. Moreover, intestinal Behçet's disease is difficult to treat and often requires surgery because of the failure of conventional medical treatment. Administration of anti-tumor necrosis factor–α, a potential therapeutic strategy, is currently under active clinical investigation, and evidence of its effectiveness for both intestinal Behçet's disease and inflammatory bowel diseases has been accumulating. Here, we review updated data on current experiences and outcomes after the administration of anti-tumor necrosis factor–α for the treatment of intestinal Behçet's disease. In addition to infliximab and adalimumab, which are the most commonly used agents, we describe agents such as golimumab, etanercept, and certolizumab pegol, which have recently been shown to be effective in refractory intestinal Behçet's disease. This review also discusses safety issues associated with anti-tumor necrosis factor–α, including vulnerability to infections and malignancy.

Cancer Risk in Patients with Intestinal Behçet’s Disease: A Nationwide Population-Based Study

BACKGROUND/AIMS: The relationship between intestinal Behçet’s disease (BD) and cancer remains unclear. We conducted a nationwide, population-based study to determine the risk of cancer in patients with intestinal BD. METHODS: Using the National Health Insurance claims records, we collected data on 365 patients who had been diagnosed with intestinal BD between 2011 and 2014. Standardized incidence ratios (SIRs) of overall and site-specific cancers in patients with intestinal BD in comparison with the general population were calculated. RESULTS: Among 167 men with intestinal BD, four cases of cancer were observed; among 191 women with BD, eight cases of cancer were observed. The risk of all cancers was significantly higher in women with intestinal BD than in women of the general population (SIR, 4.27; 95% confidence interval [CI], 1.84 to 8.41). However, in men with intestinal BD, the risk of all cancers was not significantly higher than that in men of the general population (SIR, 2.08; 95% CI, 0.57 to 5.33). The risk of hematologic cancer was significantly higher in both men and women with intestinal BD than in their counterparts in the general population (SIR, 23.90; 95% CI, 2.89 to 86.32 in men; SIR, 34.47; 95% CI, 4.17 to 124.51 in women). In particular, patients with intestinal BD showed a higher risk of leukemia and myelodysplastic syndrome than the general population. CONCLUSIONS: Patients with intestinal BD demonstrated a higher risk of hematologic cancer, especially leukemia, than the general population. Furthermore, women with intestinal BD showed a higher risk of all cancers.

Treatment and outcomes: medical and surgical treatment for intestinal Behçet's disease

Behçet's disease (BD) is a chronic relapsing disease involving multiple organ systems. BD is characterized clinically by oral and genital aphthae, cutaneous lesions, and ophthalmological, neurological, and/or gastrointestinal manifestations. It is widely recognized that the presence of intestinal lesions may be a poor prognostic factor in intestinal BD, increasing the risk of surgery and decreasing the quality of life. Despite this, the management of intestinal BD has not been standardized. Empirical therapies including 5-aminosalicylic acid and corticosteroids have been used anecdotally to treat intestinal BD, but recent studies have provided evidence for the efficacy of anti-tumor necrosis factor α monoclonal antibodies. The development of agents targeting tumor necrosis factor α continues, it seems likely that they will change the therapeutic strategy and clinical outcomes of intestinal BD and inflammatory bowel disease. Monitoring disease activity such as endoscopic evaluation will become more important to obtain better outcomes. Here, we review current and future perspectives in the treatment and outcomes of intestinal BD.

Case of Moyamoya Disease Aggravated during the Treatment of Behçet’s Disease

Behçet’s disease (BD) is a multi-organ involved inflammatory disorder described by recurrent oral ulcers and other systemic manifestations. Almost all the clinical manifestations of BD are believed to be due to vasculitis. On the other hand, the cerebral arteries are rarely involved. Moyamoya disease (MMD) is an unusual chronic cerebrovascular disorder that is described by bilateral progressive stenosis or occlusion of the internal carotid artery and an abnormal collateral vascular network. A 32-year-old woman with MMD was referred for fever, oral pain, and diarrhea, and was diagnosed with BD. Her MMD was aggravated during treatment with high dose steroids to control the intestinal BD and a reduction in the MMD medication due to gastrointestinal bleeding. This is the first reported case of intestinal BD in a patient previously diagnosed with MMD, who experienced aggravation of her MMD after the cessation of MMD medication due to aggravated intestinal BD.

Usefulness of Measuring Serum Procalcitonin Levels in Patients with Inflammatory Bowel Disease

BACKGROUND/AIMS: The relationships between serum procalcitonin, inflammatory bowel disease (IBD) and intestinal Behçet's disease (BD) have not been completely determined. We aimed to evaluate the usefulness of measuring serum procalcitonin levels to assess disease activity and infection stage in patients with IBD and intestinal BD. METHODS: We retrospectively analyzed clinical data from 129 patients with IBD and intestinal BD for whom serum procalcitonin and C-reactive protein (CRP) levels were measured between January 2006 and February 2013. RESULTS: The median serum procalcitonin levels in the IBD and intestinal BD with septic shock or sepsis (n=8), with localized infection (n=76), and without infection (n=45) were 3.46 ng/mL (range, 0.17 to 63.66 ng/mL), 0.22 ng/mL (range, 0.05 to 140.18 ng/mL), and 0.07 ng/mL (range, 0.00 to 31.50 ng/mL), respectively (p=0.001). The serum CRP levels in the IBD and intestinal BD patients did not differ according to the infection stage. Variations in serum procalcitonin levels were not observed in the IBD and intestinal BD patients with different disease activities. CONCLUSIONS: Serum procalcitonin levels may not be affected by IBD and intestinal BD activity itself, although they may be affected by concomitant infection. Serum procalcitonin measurements could be more useful than CRP in determining the infection stage that reflects the severity of infection in IBD and intestinal BD patients.

Intestinal Behcet's Disease: A True Inflammatory Bowel Disease or Merely an Intestinal Complication of Systemic Vasculitis?

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.

Clinical features analysis of 36 cases of intestinal Behcet's disease / 中华消化杂志

Objective To improve the knowledge of intestinal Behcet's disease.Methods From October 2012 to January 2014,36 patients with intestinal Behcet's disease were collected.The general information of the patients,manifestation of Behcet's disease,digestive symptoms,lab findings,imaging findings,endoscopic examination results,treatment and prognosis of Behcet's disease were analyzed.Chi square test and Fisher exact probability test were performed for count data analysis.Single factor and multi factors of efficacy were analyzed by Logistic regression analysis.Results Among 36 patients with Behcet's disease (male 19 and female 17),the mean onset age was 26.5 years old and the average course of disease was (8.2±6.8) years.Symptoms of digestive system of 91.7％ (33/36) patients showed up later than system manifestations of Behcet's disease appeared.All 36 patients had oral ulcer.The rates of genital ulcers,skin diseases,inflammation and joint disease were 63.9％ (23/36),58.3％ (21/36),25.0％ (9/36) and 25.0％ (9/36),respectively.The main symptoms of digestive system were abdominal pain,diarrhea,bloating and hemafecia,of which abdominal pain was the predominant symptom and the incidence rate was 75.0％ (27/36).Gastrointestinal bleeding,intestinal perforation,intestinal obstruction and arteriovenous fistula were the common complications of Behcet's disease and the incidence rates were 36.1％ (13/36),2.8％ (1/36),2.8％ (1/36) and 2.8％ (1/36),respectively.Among patients with ocular lesions,male patients were more than female patients (male six and female three),and the difference was statistically significant (x2 =0.111,P＜0.05).Erythrocyte sedimentation rate increased in 55.6％ (20/36) patients.C-reactive protein was positive in 27.8％ (10/36) patients.Nine patients received computed tomography (CT) examination,there were five cases with thickened bowel wall and one case with proliferative lesions at ileocecal valve.Ileocecum was the predilection site,and accounted for 36.1％ of the disease (13/36).Endoscopic appearance was mainly ulcers.Pathological examination indicated non-specific acute and chronic gastrointestinal mucosal inflammation and the formation of inflammatory granulation tissue.Twerty-two patients received sulfasalazine (SASP) treatment and 24 patients accepted glucocorticoid therapy.After Behcet's disease recurrence,five patients received combined immunosuppressive therapy,five patients received immunosuppressive therapy and tumor necrosis factor (TNF) antagonist treatment,and nine patients received TNF antagonist treatment.All the patients treated together with proton pump inhibitor (PPI).Twelve patients without glucocorticoid treatment took thalidomide and immunosuppressive therapy.The efficacy was good in patients with immunosuppressive therapy or TNF antagonist treatment and the recurrence rate was low (r=3.984,P ＜ 0.05).Conclusions Most patients with intestinal Behcet' s disease are young adults.Symptoms of digestive system show up later than system manifestations of Behcet's disease appeared.Ileocecum is the predilection site of digestive system.The common endoscopic appearance is ulcers.Corticosteroids and sulfasalazine are the main therapy,which could be much more effective when combined with tumor necrosis factor antagonists or immunosuppressive therapy.

Overlooked Management and Risk Factors for Anemia in Patients with Intestinal Behcet's Disease in Actual Clinical Practice

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.

Correlation between Soluble Triggering Receptor Expressed on Myeloid Cells-1 and Endoscopic Activity in Intestinal Behcet's Disease

PURPOSE: The serum levels of soluble triggering receptor expressed on myeloid cells-1 (sTREM-1) have recently been shown to be correlated highly with disease activity in patients with intestinal Behcet's disease (BD). However, it remains unclear whether sTREM-1 levels reflect endoscopic activity in intestinal BD. This study aimed to evaluate the correlation of sTREM-1 levels with endoscopic activity in intestinal BD. MATERIALS AND METHODS: A total of 84 patients with intestinal BD were enrolled. Endoscopic activity was compared with sTREM-1 levels as well as other laboratory findings, including erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). RESULTS: sTREM-1 levels were significantly increased in intestinal BD patients compared with controls (37.98+/-27.09 pg/mL vs. 16.65+/-7.76 pg/mL, p=0.002), however, there was no difference between endoscopically quiescent and active diseases (43.53+/-24.95 pg/mL vs. 42.22+/-32.68 pg/mL, p=0.819). Moreover, serum sTREM-1 levels did not differ in terms of number, shape, depth, size, margin, or type of ulcer in patients with intestinal BD. However, mean ESR and CRP levels in patients with active disease were significantly higher than those in patients with quiescent disease (p=0.001, p<0.001, respectively). In addition, endoscopic activity scores for intestinal BD were correlated significantly with both CRP levels (gamma=0.329) and ESR (gamma=0.298), but not with sTREM-1 levels (gamma=0.166). CONCLUSION: Unlike CRP levels and ESR, serum sTREM-1 levels were not correlated with endoscopic activity in patients with intestinal BD.

Radiation Exposure from Abdominal Imaging Studies in Patients with Intestinal Behcet Disease

BACKGROUND/AIMS: Recently, several studies have revealed that diagnostic imaging can result in exposure to harmful levels of ionizing radiation in inflammatory bowel disease patients. However, the extent of radiation exposure in intestinal Behcet disease (BD) patients has not been documented. The aim of this study was to estimate the radiation exposure from abdominal imaging studies in intestinal BD patients. METHODS: Patients with a diagnosis of intestinal BD established between January 1990 and March 2012 were investigated at a single tertiary academic medical center. The cumulative effective dose (CED) was calculated retrospectively from standard tables and by counting the number of abdominal imaging studies performed. High exposure was defined as CED >50 mSv. RESULTS: In total, 270 patients were included in the study. The mean CED was 41.3 mSv, and 28.1% of patients were exposed to high levels of radiation. Computed tomography (CT) accounted for 81.7% of the total effective dose. In multivariate analyses, predictors of high radiation exposure were azathioprine/6-mercaptopurine use, surgery, and hospitalization. CONCLUSIONS: Approximately a quarter of intestinal BD patients were exposed to harmful levels of diagnostic radiation, mainly from CT examination. Clinicians should reduce the number of unnecessary CT examinations and consider low-dose CT profiles or alternative modalities such as magnetic resonance enterography.

The Medical Treatments of Intestinal Behcet's Disease: An Update

Behcet's disease (BD) is a systemic immunological disorder characterized by recurrent mucosal ulcerative lesions including oral and genital ulcerations in association with skin and ocular involvements. BD also can involve the gastrointestinal tract. Gastrointestinal involvement of BD is one of the major causes of morbidity and mortality for this disease. However, clinical data are quite limited because of the rarity of intestinal BD. Therefore, the management of intestinal BD is heavily dependent on expert opinions and standardized medical treatments of intestinal BD are yet to be established. In this brief review, the authors summarized the currently available medical treatments such as 5-aminosalicylic acids, corticosteroids, immuno-modulators, and anti-TNF agents. Moreover, we sought to suggest a treatment algorithm for intestinal BD based on the recently published and updated data.

Outcome Predictors for Intestinal Behcet's Disease

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.

A Survey of Actual Clinical Application Patterns in Korean Diagnostic Guidelines for Inflammatory Bowel Disease / 대한소화기학회지

BACKGROUND/AIMS: The aim of this study was to analyze the actual application patterns of how Korean diagnostic guidelines for inflammatory bowel disease (IBD) were applied in clinical practice. METHODS: Questionnaires regarding guidelines for ulcerative colitis (UC), Crohn's disease (CD), intestinal Behcet's disease (BD) and intestinal tuberculosis (TB), were distributed during the 2011 Korean Association for the Study of Intestinal Disease annual conference, and e-mail survey was additionally conducted. Forty eight questionnaires were collected. RESULTS: Most of responders (79.2%) were working at secondary (> or =500 beds) or tertiary referral centers. For the necessity of guidelines, 93.8% of responders gave positive answers in UC; 95.8% in CD; 81.3% in BD; 91.7% in TB. Of the clinicians, 95.8%, 91.7%, 64.6%, 77.1% had read UC, CD, BD and TB guideline, and 87.0%, 93.2%, 90.3%, and 92.0% replied that diagnostic guidelines for UC, CD, BD and TB were helpful in practice, respectively. Practice patterns were changed in 39.1%, 33.2%, 41.9%, and 54.1% of responders by UC, CD, BD and TB guidelines, respectively. For the needs of update, 58.7% of responders answered 'yes' in UC, 54.5% in CD, 51.6% in BD and 48.7% in TB. There were differences between recommendations and practice patterns, including colonoscopy surveillance in UC, radiological examinations for small bowel in CD and for intestinal obstruction in UC, or biopsy method in UC, CD and TB, and diagnostic criteria in BD. CONCLUSIONS: Although most of responders perceived the Korean diagnostic guidelines for IBD, there were differences between recommendations of guidelines and actual practice patterns. Therefore, the publicity and revision of diagnostic guidelines are important to reconcile theory and practice.

Aplastic Anemia with Trisomy 8 and Trisomy 9 in Intestinal Behcet's Disease / 대한소화기학회지

Behcet's disease is a multisystemic inflammatory disease characterized with recurrent oral ulcer, genital ulcer, and multiple organ involvement. Aplastic anemia is one of the rarest complications of Behcet's disease. There were only several reports about Behcet's disease associated myelodysplatic syndrome worldwide. Moreover, aplastic anemia in intestinal Behcet's disease was rarely reported. Here, we present a case of aplastic anemia with trisomy 8 and trisomy 9 in intestinal Behcet's disease and a review of the literatures. To the authors' knowledge, this is the first case ever reported in Korea.

Clinical Course of Intestinal Behcet's Disease according to the Characteristics of Ulcer in Colonoscopy

BACKGROUND/AIMS: Intestinal involvement with bleeding and perforation is one of the main causes of morbidity and mortality in patients with Behcet's disease (BD); however, the clinical course of intestinal BD has not been defined. The aim of this study was to determine the clinical course of intestinal BD based on the characteristics of ulcers visualized during colonoscopy. METHODS: We retrospectively reviewed the medical records and colonoscopic findings of 41 patients with intestinal BD. All patients were classified into subgroups according to ulcer depth, size, and number, and we analyzed the clinical manifestations, subset type of BD, medications, surgical procedures, and relapse rate among the subgroups. RESULTS: The median age at the time of diagnosis was 38 years (range, 18-74 years); 25 patients were females (M:F=1:1.56). Abdominal pain (n=40), diarrhea (n=27), and RLQ tenderness (n=21) were common symptoms. The number of complete, incomplete, and suspected types was 3, 17, and 21, respectively. In an analysis according to ulcer depth, the rate of steroid use and intestinal surgeries was more frequent in the deep ulcer group compared with the aphthous and shallow ulcer groups (50.0% vs. 0% vs. 17.6%; P=0.012; 42.9% vs. 0% vs. 23.5%: P=0.013) The rate of steroid use and relapse tended to be higher as the size of the ulcer increased; however, there was no difference in the rates of steroid use, surgeries, and relapse based on the number of intestinal ulcers. CONCLUSIONS: The prognosis was worse in patients with intestinal BD who have deeper and larger ulcers. Therefore, we suggest that such patients need to be treated aggressively.

Clinical Course of Intestinal Behcet's Disease according to the Characteristics of Ulcer in Colonoscopy

BACKGROUND/AIMS: Intestinal involvement with bleeding and perforation is one of the main causes of morbidity and mortality in patients with Behcet's disease (BD); however, the clinical course of intestinal BD has not been defined. The aim of this study was to determine the clinical course of intestinal BD based on the characteristics of ulcers visualized during colonoscopy. METHODS: We retrospectively reviewed the medical records and colonoscopic findings of 41 patients with intestinal BD. All patients were classified into subgroups according to ulcer depth, size, and number, and we analyzed the clinical manifestations, subset type of BD, medications, surgical procedures, and relapse rate among the subgroups. RESULTS: The median age at the time of diagnosis was 38 years (range, 18-74 years); 25 patients were females (M:F=1:1.56). Abdominal pain (n=40), diarrhea (n=27), and RLQ tenderness (n=21) were common symptoms. The number of complete, incomplete, and suspected types was 3, 17, and 21, respectively. In an analysis according to ulcer depth, the rate of steroid use and intestinal surgeries was more frequent in the deep ulcer group compared with the aphthous and shallow ulcer groups (50.0% vs. 0% vs. 17.6%; P=0.012; 42.9% vs. 0% vs. 23.5%: P=0.013) The rate of steroid use and relapse tended to be higher as the size of the ulcer increased; however, there was no difference in the rates of steroid use, surgeries, and relapse based on the number of intestinal ulcers. CONCLUSIONS: The prognosis was worse in patients with intestinal BD who have deeper and larger ulcers. Therefore, we suggest that such patients need to be treated aggressively.

The Prevalence and Clinical Characteristics of Esophageal Involvement in Patients with Behcet's Disease: A Single Center Experience in Korea

While a significant amount of clinical information has been reported concerning intestinal involvement in Behcet's disease (BD), esophageal involvement in BD has not yet been studied extensively. The aim of this study was to evaluate the prevalence of esophageal involvement in BD and its clinical characteristics. We retrospectively reviewed the medical records of 842 patients diagnosed with BD at a single tertiary institution in Korea between January 1990 and June 2006. Of the 842 patients with BD, 129 patients (15.3%) experienced upper gastrointestinal symptoms that required inspection through esophagogastroduodenoscopy. Esophageal involvement was found in 6 (4.7%) of the 129 patients. The activity index of Behcet's disease did not differ among patients with or without esophageal involvement. All patients with esophageal involvement responded well to medical treatment and no one experienced serious complications. The results of our study demonstrate that the prevalence of esophageal involvement in BD is very low and that most patients with such involvement face few complications and respond well to medical treatment.

Diagnosis of Intestinal Behcet's Disease / 대한소화기학회지

Due to similar manifestations of intestinal Beh?et's disease (BD) to those of other colitis such as Crohn's disease or intestinal tuberculosis, it is still challenging for gastroenterologist to accurately diagnose intestinal BD in patients with ileo-colonic ulcers. Moreover, no reliable diagnostic criteria for intestinal BD have been developed yet. Therefore, IBD Study Group of KASID was formulated to establish the guideline for the diagnosis of intestinal BD using a modified Delphi process. The novel diagnostic criteria for intestinal BD were developed based on two aspects; colonoscopic findings and extra-intestinal systemic manifestations, in which patients were categorized into 4 groups including definite, probable, suspected, and non-diagnostic for intestinal BD. Furthermore, Disease Activity Index for intestinal BD was developed through a Korean multicenter study. These diagnostic and disease activity guidelines will contribute to understand intestinal BD.