Behçet’s Disease with Severe Arterial Involvement in a Child

Beh?et's disease is a vasculitis characterised by its thrombotic tendency. In some patients, manifestations of vascular lesions may dominate the clinical picture instead of the classic triad. We describe a 13-year-old boy with a 1.5-year history of Beh?et's disease presenting with complaints of acute abdominal pain, severe headache and decreased vision. His work-up revealed a saccular aneurysm of the distal abdominal aorta, thrombosis in the right common iliac, external iliac and femoral arteries, and thrombosis of the superior sagittal sinus. Aortoiliac bypass with a Dacron graft was performed with success. He also received cyclosporin A and anticoagulant therapy. Severe vasculitis may become overt at any age in patients with Beh?et's disease. Early diagnosis and management is important to prevent morbidity and mortality.     

Smoking and Behçet’s Disease

It has recently been claimed that some of the symptoms in patients with Beh?et's disease (BD) can be activated after the patient has stopped smoking. In this study we investigated the effect of smoking on the symptoms of Beh?et's disease. Fifty asymptomatic current smokers (CS) who promised to stop smoking (group 1) and 60 current non-smokers (NS) (group 2) (21 of them ex-smokers) with BD were examined at the beginning and a week later for the presence of symptoms of BD. Forty-seven of the 50 CS completed the study. Oral aphthous ulcers were observed in 31 (65.9%) of them at the end of the study period. Besides oral aphthous lesions, genital ulcers were detected in two and erythema nodosum in two other patients. Only 15 (25%) group 2 patients developed oral aphthous ulcers during the study period. The difference between the frequencies of oral aphthous lesions in these groups was significant (p=0.0002). We concluded that cessation of cigarette smoking can activate the mucocutaneous symptoms, especially oral aphthous lesions, in patients with BD.     

Crohn’s and Behçet’s Disease Association Presenting with Superior Vena Cava Thrombosis

A 21-year-old male patient with a 6-year history of Crohn’s disease presented with shoulder pain and edema in the neck and bilateral supraclavicular fossa. Color Doppler ultrasonography and computed tomography scan revealed bilateral thrombosis of the external jugular veins, brachiocephalic veins, and superior vena cava. Other etiologies were appropriately ruled out and the thrombosis was initially attributed to Crohn’s disease. In the follow-up, the patient developed recurrent, painful orogenital ulcers and acne vulgaris-like skin eruptions. HLA tissue typing was HLA B-51. The diagnosis of Crohn’s disease associated with Behçet’s disease was made. Although there are several case reports describing such an association, a Medline research revealed that this was the first presentation of such an association with thrombosis. Thrombosis in unusual sites may reflect the uncommon association of Crohn’s disease with hypercoagulable conditions such as Behçet’s disease.     

Anti-Alpha-Enolase Antibody as a Serologic Marker and Its Correlation with Disease Severity in Intestinal Behçet’s Disease

Background

Intestinal Behçet’s disease (BD) is a chronic inflammatory bowel disease, as are Crohn’s disease (CD) and ulcerative colitis (UC). But unlike CD and UC, serologic markers for intestinal BD are not well known. Recently, anti-α-enolase antibody (AAEA) has been detected in sera from BD patients.

Aims

The aim of this study was to evaluate the prevalence of AAEA in intestinal BD and its clinical correlations.

Methods

The study sample included 80 patients with intestinal BD and 23 healthy controls. IgM AAEA was detected by ELISA. The positivity of IgM AAEA was defined as an optical density greater than three standard deviations above the mean of the control sera. Other parameters, such as demographic information, subtype of BD, colonoscopic findings, disease severity and treatment modality, were analyzed retrospectively.

Results

The prevalence of IgM AAEA was 67.5% in intestinal BD and 0% in the control group. The positivity rate of IgM AAEA was higher in complete or incomplete BD than in suspected BD (77.5% vs. 51.6%, P = 0.016). The mean HBI score was higher in antibody positive patients than in antibody negative patients (5.60 vs. 4.61, P = 0.003). The cumulative probability of steroid use for aggravation of intestinal and extra-intestinal symptoms was higher in antibody positive patients than in antibody negative patients (P = 0.012). The number of patients with systemic involvement was higher in the AAEA positive group than in the negative group.

Conclusions

Monitoring IgM AAEA may be helpful for diagnosis of intestinal BD and could be used to predict clinical course and disease severity.

     

An Unusual Presentation of Behçet’s Disease: Intestinal Perforation

Beh?et's disease (BD), when first described in 1937, consisted of three symptoms: recurrent oral and genital ulcerations and iridocyclitis. Today, it is known that BD is a multisystemic chronic vasculitic disorder which may involve both arteries and veins of all sizes, as well as the central nervous and gastrointestinal systems. The rate of gastrointestinal involvement of BD varies in different populations, being more common in Japan (50%-60%) and less common in the Mediterranean basin, including Turkey (0%-5%). We present a 34-year-old Turkish woman with BD who had ileal and colonic ulcerations complicated by perforation and gastrointestinal bleeding. Special emphasis was placed on the differential diagnosis between Crohn's disease (CD) and BD with gastrointestinal involvement.     

Mucosal Healing Predicts the Long-Term Prognosis of Intestinal Behçet’s Disease

Background

Mucosal healing (MH) has emerged as a therapeutic goal in the treatment of inflammatory bowel disease; however, little is known about the impact of MH on the prognosis of intestinal Behçet’s disease (BD).

Aim

We investigated whether MH could predict the prognosis of intestinal BD.

Methods

We retrospectively reviewed the medical records of 80 patients with intestinal BD who underwent colonoscopy within 3 months after clinical remission. The clinical recurrence rate according to the presence or absence of MH was evaluated using the Kaplan–Meier method and the log-rank test. In order to evaluate MH as an independent prognostic factor, a multivariate analysis using Cox proportional hazards regression model was performed including other potential factors for the relapse of intestinal BD.

Results

The number of patients with active ulcers at the time of clinical remission was 57 (71.3 %), while 23 patients (28.7 %) were experiencing MH. In the active ulcer group, 39 patients (68.4 %) experienced recurrence during the follow-up period, whereas 7 patients (30.4 %) recurred in the MH group. The cumulative recurrence rate was significantly higher in the active ulcer group than in the MH group (P < 0.001). A multivariate analysis identified active ulcers at the time of clinical remission as an independent predictive factor for relapse.

Conclusion

Our study demonstrates that MH is an independent factor predictive of long-term prognosis of intestinal BD. MH might be the ultimate therapeutic goal in the treatment of intestinal BD.     

Anti-Saccharomyces cerevisiae Antibody in Intestinal Behçet’s Disease Patients: Relation to Clinical Course

Purpose This study was designed to assess anti-Saccharomyces cerevisiae antibody positive rate in Beh?et’s disease and intestinal Beh?et’s disease and to evaluate whether anti-Saccharomyces cerevisiae antibody expression is associated with clinical findings at diagnosis and clinical course of intestinal Beh?et’s disease. Methods One hundred six patients with intestinal Beh?et’s disease, 30 patients with Beh?et’s disease, and 45 healthy control subjects were included. Anti-Saccharomyces cerevisiae antibody was detected by indirect immunofluorescence assay. According to anti-Saccharomyces cerevisiae antibody expression, the various parameters at diagnosis, cumulative relapse rates, and cumulative probabilities of operation were analyzed. Results Anti-Saccharomyces cerevisiae antibody positive rate was 44.3 percent in intestinal Beh?et’s disease, 3.3 percent in Beh?et’s disease, and 8.8 percent in healthy control subjects. In patients with intestinal Beh?et’s disease, age, gender, distribution of Beh?et’s disease subtype, symptoms, laboratory tests, and colonoscopic findings at diagnosis were not different according to anti-Saccharomyces cerevisiae antibody expression. Cumulative probability of a first operation was significantly higher in anti-Saccharomyces cerevisiae antibody (+) intestinal Beh?et’s disease than in anti-Saccharomyces cerevisiae antibody (−) intestinal Beh?et’s disease: 44.8 and 17.2 percent at one year, and 53 and 24.3 percent at two years after diagnosis, respectively (P = 0.006). The number of patients who underwent two or more operations was higher in anti-Saccharomyces cerevisiae antibody (+) intestinal Beh?et’s disease than in anti-Saccharomyces cerevisiae antibody (−) intestinal Beh?et’s disease (21.3 vs. 8.5 percent). The cumulative relapse rates were not different between the two groups. Conclusions Anti-Saccharomyces cerevisiae antibody positive rate was 44.3 percent in intestinal Beh?et’s disease. Clinical findings at diagnosis and cumulative relapse rates of intestinal Beh?et’s disease were not found to be associated with anti-Saccharomyces cerevisiae antibody expression. However, patients with anti-Saccharomyces cerevisiae antibody (+) intestinal Beh?et’s disease were more likely to receive surgical treatment. Poster presentation at Digestive Disease Week, New Orleans, Louisiana, May 16 to 19, 2004. Supported by a faculty research grant of Yonsei University College of Medicine for 2003 (6-2003-0013).     

Prediction of Free Bowel Perforation in Patients with Intestinal Behçet’s Disease Using Clinical and Colonoscopic Findings

Background

Gastrointestinal tract involvement in Behçet’s disease (BD) often requires surgical intervention due to serious complications such as intestinal perforation, fistula formation, or massive bleeding.

Aim

The aims of this study were to investigate the clinical and surgical features of free bowel perforation and to determine the risk factors associated with this complication in intestinal BD patients.

Methods

We reviewed the medical records of 129 patients with intestinal BD treated from September 1988 to September 2008. Among them, 33 patients had intestinal perforations and all underwent emergent or elective laparotomy.

Results

The mean age of the patients with bowel perforation was 34.8 ± 15.6 years (range 12–70 years) with a sex ratio of 2.3:1 (male:female). Twenty-seven (81.8%) patients were diagnosed with intestinal BD preoperatively, whereas six (18.2%) patients were diagnosed by pathological examination after operation. Fourteen (42.4%) patients experienced postoperative recurrence of intestinal BD and 11 (33.3%) underwent reoperation. Multivariate Cox hazard regression analysis identified younger age (≤25 years) at diagnosis (HR = 3.25; 95% CI, 1.41–7.48, p = 0.006), history of prior laparotomy (HR = 5.53; 95% CI, 2.25–13.56, p = 0.0001), and volcano-shaped intestinal ulcers (HR = 2.84; 95% CI, 1.14–7.08, p = 0.025) as independent risk factors for free bowel perforation in intestinal BD.

Conclusions

According to the results of our study, patients diagnosed with intestinal BD younger than 25 years, who had a history of prior laparotomy or volcano-shaped intestinal ulcers have an increased risk of free bowel perforation.     

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

Background/Aims

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.

Methods

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.

Results

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 ± 2.50 ng/ml) was significantly higher than that in controls (1.68 ± 0.90 ng/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 ± 1.23 pg/ml) was higher than that in controls (5.40 ± 0.15 pg/ml, p = 0.009). SAA released by HT-29 cells was markedly increased by tumor necrosis factor-α (TNF-α) and lipopolysaccharides stimulation.

Conclusions

Our proteomic analysis revealed that SAA was up-regulated in intestinal BD patients.

     

Unusual enthesitis in a patient with Behçet’s syndrome

Clinical Rheumatology -     

Recurrent uveitis due to sildenafil usage in a patient with Behçet’s disease

Behçet’s disease (BD) may cause uveitis and retinal vasculitis in nearly half of the patients. Uveitis is one of the most serious complications that can lead to blindness. Sildenafil (Viagra(R)) and the other phosphodiesterase type 5 (PDE5) inhibitors are the first-line options for the treatment of erectile dysfunction, but transient visual symptoms and serious ocular side effects have been reported in PDE5 inhibitor users. Herein, we report a case with BD who applied to our outpatient unit with recurrent uveitis after sildenafil therapy.     

Intestinal Behçet’s disease with pyoderma gangrenosum successfully treated with the combination therapy of adalimumab and glucocorticoids

A 24-year-old male presented with orogenital ulcers, folliculitis, and progressive painful skin ulcers with a raised inflammatory border. Colonoscopy revealed volcano-shaped intestinal ulcers in the ascending colon, and hence, he was diagnosed as intestinal Behçets disease (BD) with pyoderma gangrenosum (PG). Treatment with systemic glucocorticoids and adalimumab dramatically improved the patient’s symptoms. Our case demonstrates that early induction of adalimumab may contribute to the successful treatment of such difficult-to-treat conditions as intestinal BD with PG.     

Ulnar artery aneurysm in a patient with Behçet’s disease

Behçet’s disease is a systemic disease characterized by oral aphthosis, genital ulcers, ocular lesions, gastrointestinal, musculoskeletal, neurological and major vessel involvement. Arterial involvement, aneurysms and arterial thrombosis have been reported in 1.5–3% of patients. In this case report, we present a patient with ulnar arterial aneurysm associated with Behçet’s disease.     

Successful treatment with adalimumab in a patient with coexisting Behçet’s disease and ankylosing spondylitis

The objective is to report a patient with concomitant ankylosing spondylitis (AS) and Behçet’s disease (BD) successfully treated with adalimumab. A 44-year-old male diagnosed as AS applied to our outpatient clinic with complaints of morning stiffness, pain and limitation of motion at spine, concurrence of oral and genital ulcerated lesions. He was on sulfasalazine together with different NSAIDs for the past 1 year. According to the criteria of International Study Group, he was diagnosed as BD. The patient was considered as refractory to current treatment and adalimumab treatment was started. During follow-up, not only AS was in remission, but also no new oral and genital ulcerations appeared. There were no complications related to the use of anti-TNFα therapy. In our case it was observed that anti-TNFα therapy, specifically adalimumab, was effective for symptoms of both AS and BD.     

Neuro-Behçet’s Syndrome in a Patient not Fulfilling Criteria for Behçet’s Disease: Clinical Features and Value of Brain Imaging

Central nervous system involvement is rarely an initial presenting manifestation of Beh?et's disease (BD). We report the case of a 33-year-old man with recurrent attacks of fever, oral mucosal ulcers, deep venous thrombosis, diplopia, vertigo and headache. Sequential brain magnetic resonance imaging (MRI) scans showed fluctuating lesions of the brain stem, mesencephalon and thalamus. F-18-fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) revealed hypometabolism at the parieto-occipital cortex at both sides and the brain stem. Treatment with prednisone and cyclosporine A led to a complete remission and normalisation of MRI and FDG-PET lesions. The present case illustrates the difficulty in the differential diagnosis of early neuro-BD.     

Huge femoral artery pseudoaneurysm in a patient with Behçet’s disease

Background

Behçet’s disease (BD) is a variable vessel vasculitis and vascular involvement is one of its life threatening manifestations. Arterial involvement frequently occurs with male predominance with pseudoaneurysms being the most common presentation. Immunosuppressive therapy is the mainstay of treatment in vascular involvement.