A patient with Behcet’s disease and IgA nephropathy in China

Successful treatment of major oral aphthous ulcers in HIV-1 infection after highly active antiretroviral therapy

SAT0574 A SYSTEMATIC LITERATURE REVIEW OF HEALTHCARE BURDEN IN PATIENTS WITH BEHÇET'S DISEASE

<h3>Background</h3> Behçet's disease (BD) is an inflammatory disease characterised by recurrent oral aphthous ulcers and numerous potential systemic manifestations. <h3>Purpose</h3> To describe the experience of our centre with the use of adalimumab, etanercept and infliximab for the treatment of severe clinical manifestations in patients with BD in whom immunosuppressive treatment had failed. <h3>Material and methods</h3> Retrospective review of medical records of 36 months (January 2010–December 2013) from patients with BD treated with adalimumab, etanercept or infliximab as compassionate use. Demographic and clinical data included age, sex, previous treatment, indication, side effects, concomitant drugs and clinical outcome. <h3>Results</h3> 12 patients were included (5/7 women/men) 3/12 treated with infliximab, 2/12 etanercept, and 7/12 adalimumab; with a mean age of 36 years (range 21–55). We decided to start treatment due to the lack of response in the control of symptoms (3/12 patients had cutaneous lesions), and ocular involvement (9/12 patients with uveitis of repetition and visual deterioration). The patients had received conventional treatment: 4/12 had received two drugs, 4/12 three drugs, 2/12 four drugs, 1/12 five drugs and one had received six drugs previously. The most prescribed drugs were corticosteroids, azathioprine and cyclosporine. 1/12 patient had received previous treatment with infliximab before adalimumab with relapse of symptoms. We did not detect any adverse effects in patients treated. In all patients, clinical improvement was evident from the first administration. 8/12 patients showed reduction of symptoms, while 4/12 patients became asymptomatic. They continue in treatment. <h3>Conclusion</h3> Anti-TNF agents are a good option for patients with severe BD who are resistant to steroid and immunosuppressive treatment, with a good safety profile. The benefit of this treatment supports the hypothesis that TNF-a is an important factor in the pathogenesis of BD. Moreover, no adverse effects were detected in the treated patients, in agreement with the few cases described in the literature reviewed. <h3>References and/or Acknowledgements</h3> No conflict of interest.

Behçet's disease (BD) is a recurrent inflammatory disease involving chronic recurrent oral aphthous ulcers (aphthae), uveitis, skin lesions and genital ulcers. We prospectively investigated the efficacy of rebamipide, a gastroprotective drug, against oral aphthous ulcers in BD patients. In a multicentre, double-blind, placebo-controlled study, 35 patients with BD, having as the main symptom oral aphthosis, were randomised to receive rebamipide 300 mg/day or placebo for 12 to 24 weeks between August 1994 and December 1996. Oral aphthosis must have occurred within 4 weeks prior to enrolment and must have been visible for at least 7 days during that time. Oral aphthae count and pain scores were recorded daily in a diary by the patients themselves. Monthly aphthae count and pain scores were defined as the sum of aphthae count and pain scores for a month, respectively. Investigators rated the global improvement in aphthae count and pain using a 6-point scale. The rate of change in monthly aphthae count and pain scores in the first 3 and last 3 months of treatment were assessed in patients with more severe symptoms whose aphthae count and pain score were >28 at baseline (trial entry). The rate of moderate or marked improvement in aphthae count and pain was 36% (5 of 14 subjects) in the placebo group and 65% (11 of 17 subjects) in the rebamipide group. During months 2 to 6 of treatment, aphthae count tended to increase and reached a peak at month 4 in the placebo group but decreased in the rebamipide group. Pain score decreased to the same extent in both groups for the first 3 months of treatment; however, in the fourth to sixth months of treatment, the pain score tended to increase in the placebo group but decreased in the rebamipide group. In patients with a monthly aphthae pain score >28 at baseline, pain and count scores decreased throughout the 6 months of rebamipide treatment but increased during the last 3 months of treatment in the placebo group (p < 0.01 for the between-group comparisons). Rebamipide is well tolerated and improves the aphthae count and pain score in BD patients. It may therefore be useful in the treatment and prevention of frequently recurrent oral aphthous ulcers (not restricted to BD). Administration of rebamipide is not cumbersome, and it does not cause any discomfort, which corticosteroid ointments for example may do; furthermore, there are no specific adverse drug reactions. Rebamipide is therefore recommended as a long-term treatment for recurrent oral aphthous ulcers.

The purpose of this study was to determine whether the application of Adlerian life style techniques would provide greater understanding of the psychological factors which shaped the life of Ernest Hemingway. Hemingway's birth order position, the dynamics and atmosphere of his family, and the early recollections of his childhood were studied and analyzed for a consistent theme or belief system. Biographies, critical evaluations of his work and selected published writing were utilized to provide a basis for a life style projection. This projection was then found to correlate with observable incidents in his adult behavior.

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OP0183 Rebamipide: A potential useful therapeutic addition in the treatment of recurrent oral ulcers in behÇet’s disease

Aphthous mouth ulcers as an initial manifestation of sécukinumab-induced inflammatory bowel disease

A causative role for Helicobacter pylori (H. pylori) in the pathogenesis of oral mucosal ulcerations has been suggested previously. We have adopted the polymerase chain reaction (PCR) as a rapid and sensitive means to detect H. pylori in swabs of recurrent oral aphthous ulcers and in samples of other oral sites. Of the oral aphthous ulcer samples, 32 (71.8%) were found to be positive, while the saliva and plaque samples (most of them taken from the patients with aphthous ulcers) were consistently negative for H. pylori DNA, as detected by the PCR assay. Only two of the swab samples from the tongue (collected at the time of concurrent, H. pylori-positive oral aphthous ulcers) were found to be positive. The data suggest that H. pylori may be associated frequently with recurrent oral aphthous ulcers, and are consistent with previous studies indicating that saliva and plaque are not likely sources of contamination with this microorganism. There was no apparent correlation with HIV status (infection with human immunodeficiency virus). The possible pathogenic significance of Helicobacter pylori in oral ulcerations is discussed.

<h3>Background</h3> Behçet9s disease (BD) is characterized by recurrent oral and/or genital aphthous ulcers accompanied by cutaneous, ocular, articular, gastrointestinal and central nervous system lesions. Diagnosing BD can be a clinical challenge in patients presenting with a limited number of organ manifestations, especially with single major organ involvement. We reported the first controlled doppler ultrasound study showing increased common femoral vein (CFV) thickness in BD (1). We recently also showed that increased CFV thickness is a distinctive feature of BD, rarely present in other inflammatory or vascular diseases such as ankylosing spondylitis, systemic vasculitides, venous insufficiency, and non-inflammatory DVT with a specificity higher than 80% for the cut-off value of ≥ 0.5 mm. We suggest that CFV thickness measurement is an easy, non-invasive diagnostic test for BD (2). <h3>Objectives</h3> In this study, we aimed to assess the diagnostic performance of CFV thickness measurement in patients with ‘Incomplete’ Behçet’s Disease diagnosed by expert opinion. <h3>Methods</h3> We included 28 patients with incomplete BD (15 male, 12 female) diagnosed with expert opinion and followed in the Marmara University Behcet’s Clinic. Demographic, clinical characteristics and treatment data were recorded during routine visits. Common femoral vein wall thickness was measured by an experienced radiologist at the same day. <h3>Results</h3> Median age was 34.3 years and median disease duration 2 years (0-16). Four patients were newly diagnosed. At follow-up onset, oral ulcers were present in 22 (78.6%), genital ulcers in 6 (21.6%), papulopustular lesions in 4 (14.3%) and pathergy positivity in 5 (17.9%) patients. Ten (35.7%) patients had familial BD. While 24 (85.7%) patients had major organ involvement, 4 (14.3%) patients had mucocutaneous disease. Distrubution of major organ involvements were given in Figure 1. All patients except 1, had CFV thickness value above the cut-off value of ≥0.5 mm. Right CFV thickness was 0.71 (0.3-1.3) mm and left CFV thickness 0.72 (0.4-1.2) mm. Bilateral femoral vein thicknesses were similar in patients with and without an history of familial BD. <h3>Conclusion</h3> Diagnosing BD can be challenging in patients presenting with one major organ involvement such as oral ulcers and posterior uveitis, brain-stem disease or arterial aneurysms, especially in countries with a low prevalence. These patients are generally diagnosed as ‘incomplete’ BD by ‘expert opinion’. Early diagnosis is of utmost importance in some of these cases, especially with venous thrombosis as their management differs from non-inflammatory venous thrombosis, necessitating immunosuppressive use rather than anticoagulant therapy. Our results show that CFV thickness measurement with Doppler US, a non-invasive, fast and cost-effective radiological modality, is a valuable diagnostic test in incomplete BD, especially with major organ involvement. <h3>References</h3> [1]Alibaz-Oner F, Ergelen R, Mutis A, Erturk Z, Asadov R, Mumcu G, et al. Venous vessel wall thickness in lower extremity is increased in male patients with Behcet’s disease. Clin Rheumatol 2019;38:1447-51. [2]Alibaz-Oner F, Ergelen R, Yildiz Y, Aldag M, Yazici A, et al. Femoral vein wall thickness measurement: A new diagnostic tool for Behcet9s disease. Rheumatology (Oxford). 2021 Jan 5;60(1):288-296 <h3>Disclosure of Interests</h3> None declared

BackgroundBehçet's disease (BD) is characterized by recurrent oral and/or genital aphthous ulcers accompanied by cutaneous, ocular, articular, gastrointestinal and central nervous system lesions. Diagnosing BD can be a clinical challenge...

<h3>Background</h3> Behçet9s disease (BD) is characterized by recurrent oral and/or genital aphthous ulcers accompanied by cutaneous, ocular, articular, gastrointestinal and central nervous system lesions. Diagnosing BD can be a clinical challenge in patients presenting with a limited number of organ manifestations, especially with single major organ involvement. We reported the first controlled doppler ultrasound study showing increased common femoral vein (CFV) thickness in BD (1). We recently also showed that increased CFV thickness is a distinctive feature of BD, rarely present in other inflammatory or vascular diseases such as ankylosing spondylitis, systemic vasculitides, venous insufficiency, and non-inflammatory DVT with a specificity higher than 80% for the cut-off value of ≥ 0.5 mm. We suggest that CFV thickness measurement is an easy, non-invasive diagnostic test for BD (2). <h3>Objectives</h3> In this study, we aimed to assess the diagnostic performance of CFV thickness measurement in patients with ‘Incomplete’ Behçet’s Disease diagnosed by expert opinion. <h3>Methods</h3> We included 28 patients with incomplete BD (15 male, 12 female) diagnosed with expert opinion and followed in the Marmara University Behcet’s Clinic. Demographic, clinical characteristics and treatment data were recorded during routine visits. Common femoral vein wall thickness was measured by an experienced radiologist at the same day. <h3>Results</h3> Median age was 34.3 years and median disease duration 2 years (0-16). Four patients were newly diagnosed. At follow-up onset, oral ulcers were present in 22 (78.6%), genital ulcers in 6 (21.6%), papulopustular lesions in 4 (14.3%) and pathergy positivity in 5 (17.9%) patients. Ten (35.7%) patients had familial BD. While 24 (85.7%) patients had major organ involvement, 4 (14.3%) patients had mucocutaneous disease. Distrubution of major organ involvements were given in Figure 1. All patients except 1, had CFV thickness value above the cut-off value of ≥0.5 mm. Right CFV thickness was 0.71 (0.3-1.3) mm and left CFV thickness 0.72 (0.4-1.2) mm. Bilateral femoral vein thicknesses were similar in patients with and without an history of familial BD. <h3>Conclusion</h3> Diagnosing BD can be challenging in patients presenting with one major organ involvement such as oral ulcers and posterior uveitis, brain-stem disease or arterial aneurysms, especially in countries with a low prevalence. These patients are generally diagnosed as ‘incomplete’ BD by ‘expert opinion’. Early diagnosis is of utmost importance in some of these cases, especially with venous thrombosis as their management differs from non-inflammatory venous thrombosis, necessitating immunosuppressive use rather than anticoagulant therapy. Our results show that CFV thickness measurement with Doppler US, a non-invasive, fast and cost-effective radiological modality, is a valuable diagnostic test in incomplete BD, especially with major organ involvement. <h3>References</h3> [1]Alibaz-Oner F, Ergelen R, Mutis A, Erturk Z, Asadov R, Mumcu G, et al. Venous vessel wall thickness in lower extremity is increased in male patients with Behcet’s disease. Clin Rheumatol 2019;38:1447-51. [2]Alibaz-Oner F, Ergelen R, Yildiz Y, Aldag M, Yazici A, et al. Femoral vein wall thickness measurement: A new diagnostic tool for Behcet9s disease. Rheumatology (Oxford). 2021 Jan 5;60(1):288-296 <h3>Disclosure of Interests</h3> None declared

Background Behcet’s disease (BD) is a rare and recurrent autoinflammatory disorder characterized by systemic vasculitis, frequently manifested as recurrent aphthous stomatitis (RAS). We aim to identify specific serum proteins to discriminate between BD and idiopathicRAS. Method Peripheral blood was collected from 12 BD patients, 12 idiopathic RAS patients, and 21 healthy volunteers. The serum samples underwent Tandem Mass Tag-based mass spectrometry analysis. Differentially expressed proteins (DEPs) were identified for KEGG pathway enrichment, Gene Ontology (GO), and protein-protein interaction (PPI) analyses. ELISA was utilized to verify two BD-specific DEPs in another cohort consisting of 18 BD patients, 18 idiopathic RAS patients, and 18 controls. Results Compared with RAS serum, BD serum showed 242 DEPs. 49 proteins were differentially expressed in BD but not RAS serum compared to healthy controls. KEGG pathway and GO analyses revealed that DEPs in BD and RAS have similar biological functions and cellular distributions, featuring a significant association with pathways regulating blood coagulation and immune response. When comparing DEPs between BD and RAS, several keratins emerged as markers that distinguish RAS from BD. We also identified multiple DEPs in BD but not RAS patients. PPI analysis uncovered that lipoprotein metabolism regulators serve as hub proteins, indicating their potentially essential roles in BD pathology. In addition, ELISA results confirmed the elevated LRG1 and SOD3 levels in BD, but not RAS patients, compared to healthy donors. Conclusion Our data uncovered novel serum proteins that distinguish BD from RAS, which may potentially be useful in BD diagnosis and treatment.

AB0082 Behcet's disease complicated with celiac trunk aneurysm

Behcet’s disease (BD) is an inflammatory disease characterized by recurrent oral aphthous ulcers and numerous systemic manifestations. The underlying cause of Behcet’s disease is unknown. As with other autoimmune diseases, the disorder may represent aberrant immune activity triggered by exposure to an antigen, perhaps infectious, in patient with a genetic predisposition to develop the disease. Here we are reporting a case of Behcet’s disease in a 50 year old gentleman who presented with fever, recurrent oral ulcers and genital ulcer. Diagnostic workup excluded other possible diseases. Pathergy test was positive. Prednisolone was started along with other supportive therapy and patient subsequently improved. Birdem Med J 2013; 3(1): 50-53 DOI: http://dx.doi.org/10.3329/birdem.v3i1.17128