Intestinal Behçet's Disease Patients Research Articles

Risk Factors for Surgery in Patients with Intestinal Behçet's Disease During Anti-Tumor Necrosis Factor-Alpha Therapy.

Behçet's disease (BD) is a chronic inflammatory immune-mediated disease involving multiorgan systems. Gastrointestinal (GI) manifestations of BD include abdominal pain, vomiting, GI bleeding, fistula formation, obstruction, and perforation that might require surgery. Recently, anti-tumor necrosis factor-alpha (anti-TNF-α) therapy has been shown to have favorable outcomes in patients with intestinal BD who are refractory to conventional therapy. This study sought to figure out the risk factors for undergoing surgery during anti-TNF-α therapy in patients with intestinal BD. In this retrospective analysis of intestinal BD patients who were treated with anti-TNF-α, we collected the baseline patient data including comorbidities, clinical, endoscopic, and radiologic characteristics, and the Disease Activity Index for Intestinal Behçet's Disease at the time of anti-TNF-α initiation. Each potential risk factor was compared. For multivariate analysis, Cox regression was used. A total of 62 patients were considered eligible for analysis, and 15 of them (24.1%) underwent surgery. In univariate analysis, the presence of extraintestinal manifestation, such as joint symptoms and erythrocyte sedimentation rate (ESR), were significantly associated with surgery during therapy. In multivariate analysis, drug response within 4 weeks [hazard ratio (HR), 64.59], skin and joint manifestation (HR, 10.23 and HR, 6.22), geographic ulcer (HR, 743.97), and ESR >42.5 mm/h (HR, 9.16) were found to be factors predictive of undergoing surgery during anti-TNF-α therapy. We found five risk factors predictive of surgery in patients with intestinal BD receiving anti-TNF-α therapy, which can guide physicians in selecting appropriate patients between anti-TNF-α therapy and early surgery.

Elevated fecal calprotectin and lactoferrin associated with small intestinal lesions in patients with Behçet disease.

Small intestinal lesions in patients with Behçet disease (BD) have a risk of perforation and hemorrhage requiring surgery. However, no screening strategy for such lesions has been established. We investigated small intestinal lesions in BD patients with video capsule endoscopy (VCE) and analyzed clinical characteristics to identify noninvasive biomarkers of such lesions. This study included 33 BD patients who underwent VCE (PillCam® SB3) at our institution from June 2016 to January 2019. Clinical characteristics, including age, sex, disease duration, body mass index, gastrointestinal symptoms, eye involvement, and blood examinations, were obtained from the medical records of 27 of the 33 patients. Fecal immunochemical tests for hemoglobin, fecal calprotectin (FC), and fecal lactoferrin (FL) were measured. VCE findings of 145 healthy Japanese individuals from a previous report were used as controls. Two intestinal BD patients were included in the 27 patients. We observed that BD patients exhibit more small intestinal lesions compared with healthy individuals, including erosions, ulcers, and total lesions (erosions or ulcers). FC and FL levels were significantly higher in patients with versus without small intestinal lesions (P=0.034 and P=0.046, respectively). Receiver operating characteristic analyses demonstrated that FC (cutoff value=119μg/g) and FL (cutoff value=17μg/g) were biomarkers for small intestinal lesions in patients with BD. The present study using VCE showed that patients with BD had more small intestinal lesions than healthy individuals. FC and FL could be useful for screening BD patients who may have small intestinal lesions.

Incidence and risk factors for anemia among newly-diagnosed intestinal Behcet's disease patients

BackgroundTo investigate the incidence, characteristics, and potential risk factors of anemia in patients with newly-diagnosed intestinal Behcet's disease (BD). MethodsIn this cross-sectional study, 106 newly-diagnosed intestinal BD patients were identified, and a gender-, age- and organ involvement-matched control group of 241 non-intestinal BD patients was established. Hemoglobin (Hb) levels below 120 g/L in women and 130 g/L in men were diagnosed as anemia; these were further classified as mild (Hb ≥ 90 g/L), moderate (60 g/L ≤ Hb < 90 g/L), and severe (Hb < 60 g/L) anemia for both genders. The prevalence, type and severity of anemia in these patients were assessed. Logistic regression was performed to analyze the relationship between clinical variables and anemia in newly-diagnosed intestinal BD patients. ResultsThe prevalence of anemia was 60.38% in newly-diagnosed patients with intestinal BD, significantly higher than those with non-intestinal BD (27.80%). Patients with intestinal BD had lower Hb, higher levels of C-reactive protein (CRP) and higher erythrocyte sedimentation rates (ESR) than did patients with non-intestinal BD (P < 0.05). The majority of patients had mild-to-moderate anemia. The most common type of anemia found in both groups was normocytic normochromic anemia (56.25% for intestinal BD and 59.70% for non-intestinal BD). Multivariate logistic regression showed that the independent risk factors for anemia were disease activity index (DAIBD) (OR = 4.949, 95% CI: 1.504–16.282), higher levels of ESR (OR = 1.058, 95% CI: 1.019–1.099), and lower body mass index (BMI) (OR = 0.843, 95% CI: 0.727–0.977) for newly-diagnosed intestinal BD patients. ConclusionAnemia is common in patients with newly-diagnosed intestinal BD. Although typically mild or moderate, anemia may closely relate with disease activity.

Differentiation between intestinal Behçet’s disease and Crohn’sdisease based on endoscopy

Background/aim Differentiating intestinal Behçet’s disease (BD) from Crohn’s disease (CD) is highly challenging, as they often mimic each other in terms of clinical manifestations. Endoscopy is an important modality for distinguishing bowel lesions. The study was designed to identify clinical manifestations that are easily confused and to evaluate the efficacy of endoscopy for distinguishing intestinal BD from CD by several overlapping signs.Materials and methods The data from 111 patients with intestinal BD and 81 patients with CD were retrospectively analyzed. Logistic regression was applied to establish a prediction model based on endoscopic findings for the differential diagnosis. The diagnostic efficacy of endoscopy was verified using the area under the receiver operating characteristic (ROC) curve.Results Among intestinal BD patients mucocutaneous lesions were the leading clinical manifestations. Gastrointestinal symptoms were common in CD but were rare in intestinal BD (P < 0.001). CD patients with moderate-to-severe activity were more common than intestinal BD patients presenting with equivalent activity (P < 0.05). Independent factors that distinguished intestinal BD from CD were solitary ulcer in the ileocecal area (P < 0.001), perianal abscess (P = 0.049), single segment (P < 0.001), round intestinal ulcer (P = 0.013), intestinal obstruction (P = 0.035), and fistula (P < 0.001). The scores ranged from –2 to 3. The area under the ROC curve was 0.874 (95% CI: 0.823–0.926) (P < 0.001). With a score of 1.5 as the diagnostic cutoff value, the sensitivity and specificity were 76.3% and 80.6%, respectively.Conclusion Mucosal injuries were rarer in patients with intestinal BD than in those with CD. The differentiation model combining several endoscopy features appeared to be reliable for distinguishing between intestinal BD and CD.

Heat shock protein family A member 6 combined with clinical characteristics for the differential diagnosis of intestinal Behçet's disease.

To investigate the role of heat shock protein family A member 6 (HSPA6) expression alone and in combination with clinical characteristics in distinguishing intestinal Behçet's disease (BD) from Crohn's disease (CD) with ileocolonic involvement. Patients diagnosed with either intestinal BD or CD were enrolled. Their clinical characteristics, disease activity, laboratory test results including hypersensitive C-reactive protein (hsCRP) and erythrocyte sedimentation rate (ESR), endoscopic, pathological and radiological features were retrospectively analyzed. Enzyme-linked immunosorbent assay was applied to measure serum HSPA6 levels. Among intestinal BD patients, abdominal pain and diarrhea were the leading gastrointestinal symptoms. Submucosal lymphocyte infiltration was the most common pathological finding. Computed tomography enterography features involved number of segments of less than 4 and bowel wall thickening. Independent factors were round/ellipsoid intestinal ulcer (P < 0.001), number of ulcers ≤5 (P = 0.050), elevated ulcer margin (P = 0.019), absence of aphthous ulcer (P = 0.005), bowel wall thickening >13 mm (P < 0.001) and serum HSPA6 level >3.725 ng/mL (P = 0.008) for the differential diagnosis between intestinal BD and CD. Serum HSPA6 expression was significantly elevated in intestinal BD (0.72 ± 0.39 ng/mL) compared with CD (0.50 ± 0.24 ng/mL, P = 0.000) and healthy controls (0.38 ± 0.37 ng/mL, P = 0.000). HSPA6 in combination with clinical, radiological and pathological characteristics is useful in distinguishing intestinal BD from CD with ileocolonic involvement.

Antibodies against glycoprotein 2 display diagnostic advantages over ASCA in distinguishing CD from intestinal tuberculosis and intestinal Behçet's disease.

Objectives:There is an increasing need to identify reliable biomarkers for distinguishing Crohn’s disease (CD) from other gastrointestinal disorders sharing similar clinical and pathological features. This study aimed at evaluating the diagnostic potential of antibodies to zymogen granule glycoprotein GP2 (aGP2) in a large, well-defined Chinese cohort with a special focus on their role in discriminating CD from intestinal Behçet's disease (BD) and intestinal tubercolosis (ITB).Methods:A total of 577 subjects were prospectively enrolled, including 171 patients with CD, 208 patients with ulcerative colitis (UC), 71 with BD, 57 with ITB and 70 healthy controls (HC). aGP2 and anti-Saccharomyces cerevisiae antibodies (ASCA) were determined by ELISA. Perinuclear antineutrophil cytoplasmic antibodies were tested by indirect immunofluorescent assay.Results:aGP2 IgG and IgA levels were significantly elevated in patients with CD compared with those in patients with UC, intestinal BD, and ITB and HC. Conversely, ASCA IgG levels were not different between CD and intestinal BD patients, whereas ASCA IgA levels did not discriminate CD from intestinal BD and ITB patients. aGP2 IgA and IgG displayed a better assay performance (larger areas under the curve) over ASCA IgA and IgG in differentiating CD from disease controls (P<0.05). ASCA IgA did not discriminate CD from disease controls. aGP2 IgA and/or IgG was significantly associated with penetrating disease (B3) and ileal CD (L1) (P<0.05), whereas ASCA IgA and/or IgG was not.Conclusions:In comparison with ASCA, aGP2 distinguishes CD from intestinal BD or ITB as disease controls more efficiently, aiding in the differential diagnosis of IBD.

Updated treatment strategies for intestinal Behçet's disease.

Behçet’s disease (BD) is a chronic, idiopathic, relapsing immune-mediated disease involving multiple organs, and is characterized by recurrent oral and genital ulcers, ocular disease, gastrointestinal ulcers, vascular diseases, and skin lesions. In particular, gastrointestinal involvement in BD is followed by severe complications, including massive bleeding, bowel perforation, and fistula, which can lead to significant morbidity and mortality. However, the management of intestinal BD has not yet been properly established. Intestinal BD patients with a severe clinical course experience frequent disease aggravations and often require recurrent corticosteroid and/or immunomodulatory therapies, or even surgery. However, a considerable number of patients with intestinal BD are often refractory to conventional therapies such as corticosteroids and immunomodulators. Recently, there has been a line of evidence suggesting that biologics such as infliximab and adalimumab are effective in treating intestinal BD. Moreover, new biologics targeting proteins other than tumor necrosis factor α are emerging and are under active investigation. Therefore, in this paper, we review the current therapeutic strategies and new clinical data for the treatment of intestinal BD.

Proteomic Analysis of Serum Amyloid A as a Potential Marker in Intestinal Behçet's Disease.

Data regarding biomarkers to understand disease pathogenesis and to assess disease activity of intestinal Behçet's disease (BD) are limited. Therefore, we aimed to investigate the differentially expressed proteins in sera from patients with intestinal BD and to search for biomarkers using mass spectrometry-based proteomic analysis. Serum samples were pooled for the screening study, and two-dimensional electrophoresis (2-DE) was performed to characterize the proteins present in intestinal BD patients. Candidate protein spots were identified using matrix-assisted laser desorption/ionization tandem time-of-flight mass spectrometry (MALDI-TOF/TOF MS) and bioinformatic analysis. To validate the proteomic results, serum samples from an independent cohort were assessed by enzyme-linked immunosorbent assay. Pooled serum samples were used for 2-DE, and approximately 400 protein spots were detected in the sera of intestinal BD patients. Of the 22 differentially expressed proteins, 3 were successfully identified using MALDI-TOF/TOF MS. The three up-regulated proteins identified in the intestinal BD group included fibrin, apolipoprotein A-IV, and serum amyloid A (SAA). Serum SAA in intestinal BD patients (2.76±2.50ng/ml) was significantly higher than that in controls (1.68±0.90ng/ml, p=0.007), which is consistent with the proteomic results. In addition, the level of IL-1β in patients with intestinal BD (8.96±1.23pg/ml) was higher than that in controls (5.40±0.15pg/ml, p=0.009). SAA released by HT-29 cells was markedly increased by tumor necrosis factor-α (TNF-α) and lipopolysaccharides stimulation. Our proteomic analysis revealed that SAA was up-regulated in intestinal BD patients.

Long-Term Outcomes and Predictors of Sustained Response in Patients with Intestinal Behcet's Disease Treated with Infliximab.

Intestinal Behcet's disease (BD) is a specific subtype of BD. Effective drug therapy for intestinal BD remains elusive. To investigate long-term outcomes and identify predictors of sustained response in intestinal BD patients receiving infliximab (IFX) treatment. The medical records were reviewed of patients received IFX from September 2012 to March 2016. The cumulative probabilities of sustained response were calculated using the Kaplan-Meier. Predictor factors for sustained response were accessed by receiver operating characteristic curve. Totally, 27 active intestinal BD patients were enrolled. Sustained responses were observed in 17 patients, after a median follow-up duration 24months (interquartile range 9-37). The proportion of clinical remission at week 14, 30, and 52 had occurred in 84.6, 70, and 70%, respectively, with the proportion of clinical remission of 69.2, 40, and 55%. The mucosal healing (MH) rate at week 14 was 72%. Kaplan-Meier estimated patients with achievement of clinical and biological responses at week 14 or MH was likely to remain sustained clinical response. ROC curve analysis revealed CRP level (of 6.85mg/L) at week 14 is a potential predictor for discriminating patients with sustained response from relapse, with an area under the curve values of 0.837. IFX is effective and safe for induction and maintenance therapy in Chinese patients with moderate-to-severe active intestinal BD. Early achievement of clinical response and mucosal healing might associate long-term response. A lower CRP level seems to be associated with a more benign clinical course.

Long-term safety and efficacy of adalimumab for intestinal Behçet's disease in the open label study following a phase 3 clinical trial.

Background/AimsIntestinal Behçet's disease (BD) is an immune-mediated inflammatory disorder. We followed up the patients and evaluated safety profile and effectiveness of adalimumab for the treatment of intestinal BD through 100 weeks rolled over from the 52 week clinical trial (NCT01243671).MethodsPatients initiated adalimumab therapy at 160 mg at week 0, followed by 80 mg at week 2, followed by 40 mg every other week until the end of the study. Long-term safety and all adverse events (AEs) were examined. The efficacy was assessed on the basis of marked improvement (MI) and complete remission (CR) using a composite efficacy index, which combined global gastrointestinal symptoms and endoscopic assessments.ResultsTwenty patients were enrolled in this study; 15 patients received adalimumab treatment until study completion. The incidence of AEs through week 100 was 544.4 events/100 person-years, which was comparable to the incidence through week 52 (560.4 events/100 person-years). No unexpected trend was observed and adalimumab was well tolerated. At weeks 52 and 100, 60.0% and 40.0% of patients showed MI, respectively, and 20.0% and 15.0% of patients showed CR, respectively.ConclusionsThis report demonstrates 2 years safety and effectiveness of adalimumab in intestinal BD patients. Patients with intestinal BD refractory to conventional treatment receiving up to 2 years of adalimumab treatment demonstrated safety outcomes consistent with the known profile of adalimumab, and the treatment led to sustained reduction of clinical and endoscopic disease activity.

Risk factors and outcomes of acute lower gastrointestinal bleeding in intestinal Behçet’s disease

Intestinal Behçet's disease (BD) can cause acute lower gastrointestinal bleeding, which is sometimes fatal. We aimed to identify the risk factors and outcomes of acute lower gastrointestinal bleeding and factors associated with rebleeding in intestinal BD patients. Of the total of 588 intestinal BD patients, we retrospectively reviewed the medical records of 66 (11.2%) patients with acute lower gastrointestinal bleeding and compared them with those of 132 matched patients without bleeding. The baseline characteristics were comparable between the bleeding group (n=66) and the non-bleeding group (n=132). On multivariate analysis, the independent factors significantly associated with lower gastrointestinal bleeding were older age (>52years) (hazard ratio [HR] 2.2, 95% confidence interval [CI] 1.058-4.684, p=0.035) and a nodular ulcer margin (HR 7.1, 95% CI 2.084-24.189, p=0.002). Rebleeding occurred in 23 patients (34.8%). Female patients (p=0.044) and those with previous use of corticosteroids or azathioprine (p=0.034) were more likely to develop rebleeding. On multivariate analysis, only use of steroids or azathioprine was significantly associated with rebleeding (HR 3.2, 95% CI 1.070-9.462, p=0.037). Age >52years and the presence of a nodular margin of the ulcer were found to be related to increased risk of bleeding in patients with intestinal BD. Rebleeding is not uncommon and not effectively prevented with currently available medications. Further studies are warranted to identify effective measures to decrease rebleeding in intestinal BD.

Clinical value of capsule endoscopy for detecting small bowel lesions in patients with intestinal Behçet's disease.

To evaluate the clinical value of capsule endoscopy (CE) in patients with intestinal Behçet's disease (BD). The present study was a case-control pilot study conducted in intestinal BD patients and healthy volunteers. A total of 19 patients with intestinal BD (intestinal BD group) and 19 healthy volunteers (control group) matched for age and sex were enrolled. Frequency, number of small bowel lesions per subject, and Lewis score were comparatively evaluated between the two groups. Of the 19 patients with intestinal BD, 18 (94.7%) had reddened lesions, 15 (78.9%) had erosions, and nine (47.4%) had ulcers. There were significant differences in the frequency of reddened lesions (P < 0.0001), erosions (P < 0.0001) and ulcers (P = 0.0011) between the two groups. The difference in the number of small bowel lesions between the two groups was also statistically significant. The median Lewis score in the intestinal BD group was significantly higher than that in the control group (intestinal BD group 237 (0-768) vs. control group 8 (0-135); P < 0.0001). Analysis according to the location in the small bowel revealed that the frequency of ulcers tended to increase towards the distal intestine. This is the first CE study conducted to examine small bowel involvement in intestinal BD patients. Our results suggest that CE evaluation is necessary, in addition to colonoscopy, in all intestinal BD patients.

Postoperative Effects of Thiopurines in Patients with Intestinal Behçet's Disease.

No studies have heretofore specifically focused on the efficacy of postoperative thiopurine therapy in intestinal Behçet's disease (BD). We conducted this study to assess the clinical effects of postoperative thiopurines in patients with intestinal BD. We reviewed the medical records of all patients with intestinal BD who had undergone bowel resection surgery in a single tertiary academic medical center between 1991 and 2013. The cumulative probabilities of clinical recurrence were calculated using the Kaplan-Meier method, and predictive factors for recurrence were assessed by multivariate analysis. A total of 77 patients with intestinal BD received 5-ASA (n = 50, 64.9%) or thiopurine (n = 27, 35.1%) therapy after surgery at our center. The postoperative recurrence rate was lower in patients who received postoperative thiopurines (P = 0.050). The hazard ratio for recurrence was 0.636 (95% confidence interval 0.130-1.016, P = 0.053) for postoperative thiopurine use compared with postoperative 5-ASA. However, the rates of re-operation, re-admission, and death were not significantly different between the 5-ASA and thiopurine groups. Thiopurine therapy after surgery exhibited a modest effect in preventing recurrence in intestinal BD patients. More patients, long-term follow-up, and a randomized controlled design are necessary to validate the effectiveness of postoperative thiopurines in patients with this disease.

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

Background/AimsAnemia 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 Behçet’s disease (BD) in actual clinical practice.MethodsWe 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.ResultsAnemia 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 Behçet’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 (≥40; odds ratio, 4.08; 95% confidence interval, 1.21 to 13.71; p=0.023).ConclusionsAlthough 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 Behçet's disease.

PurposeThe 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 Behçet'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 MethodsA 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).ResultssTREM-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 (γ=0.329) and ESR (γ=0.298), but not with sTREM-1 levels (γ=0.166).ConclusionUnlike 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 Behçet disease.

Background/AimsRecently, 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.MethodsPatients 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.ResultsIn 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.ConclusionsApproximately 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.

Clinical Course of Intestinal Behcet’s Disease During the First Five Years

Little is known about the clinical course of patients with intestinal Behcet's disease (BD). We aimed to evaluate the clinical course of intestinal BD during the first 5 years after diagnosis, and to identify factors that could predict the 5-year clinical course. We reviewed the medical records of 130 intestinal BD patients who were regularly followed-up for at least 5 years at a single tertiary academic medical center between March 1986 and September 2011. Of the five different clinical course patterns that we observed, persistent remission or mild clinical activity was the most frequent course (56.2 %). The majority of patients (74.6 %) had remission or mild clinical activity at 5 years, and only the minority (16.2 %) had multiple relapses or chronic symptoms. The clinical course of the first year after diagnosis of intestinal BD influenced the clinical course of the following years. Patients in the severe clinical course group were younger, and had a higher ESR, CRP level, and disease activity index for intestinal Behcet's disease (DAIBD), and lower albumin level at diagnosis than patients in the mild clinical course group. Initial presentation with a high DAIBD was independently associated with a severe clinical course. The clinical course of intestinal BD during the first 5 years was variable. A substantial proportion of patients went into remission or had a mild clinical activity, while some patients had a severe, debilitating clinical course as time progressed. High disease activity at diagnosis was a negative prognostic predictor.

Long-term Clinical Outcomes of Crohnʼs Disease and Intestinal Behcetʼs Disease

Crohn's disease (CD) and intestinal Behcet's disease (BD) are transmural inflammatory diseases with fluctuating courses characterized by repeated episodes of relapse and remission that often require operation or reoperation. However, no study has directly compared the long-term prognoses of these two diseases. We reviewed the medical records of 332 patients with CD and 276 patients with intestinal BD who were regularly followed up at a single tertiary academic medical center in Korea between March 1986 and July 2010. The clinical outcomes after diagnosis and surgery were analyzed using the Kaplan-Meier method and log-rank test. There were no significant differences in the cumulative probabilities of surgery (29.4% and 36.0% vs. 31.6% and 44.4% at 5 and 10 years, respectively: P = 0.287) or admission (66.1% and 73.8% vs. 59.0% and 69.2%, P = 0.259) between CD and intestinal BD. Furthermore, no differences were observed between the two diseases for the cumulative probabilities of postoperative clinical recurrence (P = 0.724) and reoperation (P = 0.770). However, the cumulative probabilities of corticosteroid use (63.8% and 76.6% vs. 42.6% and 59.4% at 5 and 10 years, respectively: P < 0.001) and immunosuppressant use (49.1% and 65.5% vs. 27.1% and 37.7%, P < 0.001) were significantly higher in CD patients than in intestinal BD patients. There were no significant differences in the long-term clinical outcomes and postoperative prognoses between CD and intestinal BD, although CD patients required corticosteroid or immunosuppressant therapy more often than intestinal BD patients.

Correlations between endoscopic and clinical disease activity indices in intestinal Behcet's disease

To develop a novel endoscopic severity model of intestinal Behcet's disease (BD) and to evaluate its feasibility by comparing it with the actual disease activity index for intestinal Behcet's disease (DAIBD). We reviewed the medical records of 167 intestinal BD patients between March 1986 and April 2011. We also investigated the endoscopic parameters including ulcer locations, distribution, number, depth, shape, size and margin to identify independent factors associated with DAIBD. An endoscopic severity model was developed using significant colonoscopic variables identified by multivariate regression analysis and its correlation with the DAIBD was evaluated. To determine factors related to the discrepancy between endoscopic severity and clinical activity, clinical characteristics and laboratory markers of the patients were analyzed. A multivariate regression analysis revealed that the number of intestinal ulcers (≥ 2, P = 0.031) and volcanoshaped ulcers (P = 0.001) were predictive factors for the DAIBD. An endoscopic severity model (Y) was developed based on selected endoscopic variables as follows: Y = 47.44 + 9.04 × non-Ileocecal area + 11.85 × ≥ 2 of intestinal ulcers + 5.03 × shallow ulcers + 12.76 × deep ulcers + 4.47 × geographic-shaped ulcers + 26.93 × volcano-shaped ulcers + 8.65 × ≥ 20 mm of intestinal ulcers. However, endoscopic parameters used in the multivariate analysis explained only 18.9% of the DAIBD variance. Patients with severe DAIBD scores but with moderately predicted disease activity by the endoscopic severity model had more symptoms of irritable bowel syndrome (21.4% vs 4.9%, P = 0.026) and a lower rate of corticosteroid use (50.0% vs 75.6%, P = 0.016) than those with severe DAIBD scores and accurately predicted disease by the model. Our study showed that the number of intestinal ulcers and volcano-shaped ulcers were predictive factors for severe DAIBD scores. However, the correlation between endoscopic severity and DAIBD (r = 0.434) was weak.

Clinical outcomes and prognostic factors for thiopurine maintenance therapy in patients with intestinal Behcetʼs disease

To date, there have been no studies focusing on the efficacy of thiopurine therapy in intestinal Behcet's disease (BD). We conducted this study to investigate clinical outcomes and predictors of clinical relapse in intestinal BD patients receiving thiopurine maintenance therapy. We reviewed the medical records of all patients with intestinal BD who received thiopurine therapy in a single tertiary academic medical center between March 1986 and October 2010. The cumulative probabilities of clinical relapse after remission were calculated using the Kaplan-Meier method. Predictors of clinical relapse were identified by univariate analysis using the log-rank test and by multivariate analysis using Cox proportional hazards regression models. Of a total of 272 patients with intestinal BD, 67 (24.6%) received their first course of thiopurine therapy at our center. Thirty-nine (58.2%) of the 67 patients constantly received thiopurines for maintaining medically or surgically induced remission. The cumulative relapse rates at 1 year, 2 years, 3 years, and 5 years after remission were 5.8%, 28.7%, 43.7%, and 51.7%, respectively. On multivariate analysis, a younger age (<25 years) at diagnosis and a lower hemoglobin level (<11 g/dL) were independent predictive factors for relapse in intestinal BD patients receiving thiopurine maintenance therapy. Thiopurine therapy showed a relatively good effect for maintenance of remission in intestinal BD patients. However, a younger age at diagnosis and a lower hemoglobin level were associated with a poor response to thiopurines, necessitating early adoption of effective alternative therapeutic options in these risk groups.