Behcet’s Disease Following Bariatric Surgery: A Case Report

AB0082 Behcet's disease complicated with celiac trunk aneurysm

To the Editor, Myelodysplastic syndrome (MDS) is a heterogeneous group of stem cell disorders of unknown etiology. Behcet disease (BD) is a systemic autoimmune vasculitis also of unknown cause. Several recent reports have suggested an association between MDS and BD [1], possibly related to a cytogenetic abnormality such as trisomy 8 [2]. In addition, some distinctive clinical characteristics have been identif ied that include a high frequency of intestinal BD, generally high disease activity, and a trend toward treatment with more potent immunosuppressive therapy [2,3]. The treatment of MDS-related Behcet colitis is usually diff icult to manage, and many patients eventually die of infection or hemorrhage. We herein report a case of intractable MDS-related Behcet colitis that was successfully treated by allogeneic hematopoietic stem cell transplantation (HSCT). A 38-year-old female was admitted to our hospital with complaints of recurrent abdominal pain and fever. In June 2001, she was diagnosed with BD according to the diagnostic criteria of the international BD study group [4]. She had bouts of several symptoms compatible with BD, such as recurrent nasal ulcers, oral ulcers, genital ulcers, migrating arthralgia, and uveitis. Total colonoscopy performed to evaluate the recurrent abdominal pain revealed a shallow ulceration of 2 × 2 cm with irregular margins in the ileocecal area (Fig. 1A). Pathologic examination showed chronic ulcerative inflammation with lymphocyte infiltration compatible with Behcet colitis (Fig. 2). She was followed closely at the rheumatology clinic and initially treated with corticosteroids alone; however, this treatment produced no relief of symptoms. Azathioprine was added, but she continued to experience recurrent abdominal pain. Sulfasalazine was administered without improvement. In spite of active management of Behcet colitis, the abdominal pain persisted in a waxing and waning manner. In April 2005, her complete blood count (CBC) values gradually decreased, particularly the leukocyte count (2.86 × 103/µL) and hemoglobin level (8.3 g/dL). In May 2008, the CBC showed further decreases in the leukocyte count (1.70 × 103/µL) and hemoglobin level (5.1 g/dL), and thrombocytopenia (90.0 × 103/µL) was detected. Bone marrow examination revealed trilineage dysplasia with less than 5% blasts. Chromosomal analysis of bone marrow aspirates showed trisomy 8. She was diagnosed with MDS with refractory cytopenia with multilineage dysplasia. According to the International Prognostic Scoring System, the disease was classified as intermediate-1. For further evaluation and therapeutic options, she was referred to our hematology clinic. She started treatment with decitabine because she did not have a human leukocyte antigen (HLA)-matched sibling donor. However, recurrent infections developed with complications such as neutropenic fever, pneumonia, and hepatosplenic candidiasis. Decitabine was stopped and hematologic supportive care begun with intermittent red cell transfusion at intervals of ~2 weeks for 15 months. We had intermittently been searching for unrelated donors from the public blood and marrow donation program since the diagnosis of MDS. Finally, a fully matched HLA-compatible unrelated donor was found in January 2010. Until that time, recurrent oral ulcers, migrating arthralgia, and abdominal pain had persisted, and she often required several days of hospitalization to modulate the pain and associated symptoms such as diarrhea. The patient underwent allogeneic peripheral blood stem cell transplantation with a myeloablative regimen in April 2010. The pretransplantation conditioning regimen comprised busulfan and cyclophosphamide. An infusion of 4.6 × 106/kg CD34+ cells was introduced. Methotrexate and cyclosporine were used routinely for graft-versus-host disease (GVHD) prophylaxis. On day 12, leukocyte engraftment was observed. Cyclosporine was discontinued on day 189, and abdominal pain was markedly diminished. On day 229 after transplantation, total colonoscopy was repeated. The previous lesion was greatly improved, and only a small shallow ulceration was observed (Fig. 1B). There was no need to treat for Behcet colitis. At the time of this report, the patient was doing well in complete remission of MDS without evidence of chronic GVHD and no signs or symptoms of BD. Figure 1 (A) Friable erythematous mucosa and edematous shallow ulceration with irregular margins were observed at the ileocecal valve. (B) Marked improvement in the previous ulcerative lesion was noted at the ileocecal valve. Only a small ulceration remained. Figure 2 Pathologic examination of the colon reveals ulceration with glandular distortion, widening of the distance between the crypts, and prominent infiltration of inf lammatory cells, compatible with Behcet colitis (H&E, × 100). MDS is an acquired clonal disorder of hematopoietic progenitor cells. Several recent studies showed that an autoimmune mechanism plays an important role in the development of MDS, and another study suggested that transformation of normal stem cells induces an autoimmune T cell response. Although MDS and BD have been regarded as different manifestations, the same pathophysiological processes, such as abnormal neutrophil function, overproduction of inf lammatory cytokines, and immunological abnormalities, are thought to be related to MDS and BD [1]. MDS-related BD shows distinctive characteristics [2,3], notably cytogenetic abnormalities such as trisomy 8 in bone marrow cells. Especially in Korea and Japan, bone marrow failure is frequently reported in patients with BD, and the proportion with trisomy 8 is strikingly high (63.6% to 86.0%) [2,3]. Clinically, BD patients with MDS have lower leukocyte counts, hemoglobin levels, and platelet counts and significantly higher levels of serum C-reactive protein and frequencies of intestinal BD than do BD patients without MDS. That is, patients with both BD and MDS have higher levels of disease activity than do BD patients without MDS. As a result, patients with MDS-related BD may be treated more frequently with corticosteroids and other immunosuppressive agents in various combinations. However, the disease is often refractory to these treatments. Furthermore, the control of intestinal BD in association with MDS using immunosuppressive agents, such as prednisolone alone, is difficult, and many patients die due to infection or hemorrhage. More encouraging are recent reports of successful treatment of MDS-related BD using HSCT. The positive response of the disorder to HSCT may plausibly occur through immunological reconstruction. A very grave risk to consider in treating MDS-related BD with HSCT is the induction of gastrointestinal GVHD. However, no reports to date have indicated that life-threatening gastrointestinal effects develop in patients treated with HSCT, including in our patient. In addition, patients with gastrointestinal disease before HSCT do not show an increased risk of gastrointestinal GVHD after treatment. Autologous HSCT even improves intestinal BD that was refractory to medical therapy [5]. In conclusion, BD may accompany MDS, possibly as an expression of a cytogenetic abnormality such as trisomy 8. A high frequency of intestinal BD with high disease activity is observed in MDS-related BD, and the intestinal disease may be refractory to therapy. HSTC presents a potentially effective treatment for MDS-related BD intractable to medical therapy without increasing the risk of gut GVHD. Further study of this treatment approach is justified.

Introduction - Behcet disease is an auto-inflammatory systemic vasculitis. It usually comprises a triad of recurrent oral and genital ulcerations, ocular manifestations. Case Report- A18 years old lady presented with recurrent oral ulceration since 1 year which was causing difficulty in chewing food and burning sensation in oral cavity. Out of high grade of clinical suspicion, due to the recurrent nature of ulcers and rapid resolution by steroids, HLA profiling was done, and HLA B5 came out to be positive and the patient was diagnosed as Behcet disease. Conclusion- Behcet disease has a multi-systemic involvement. The diagnosis of Behcet disease requires a very high threshold of clinical suspicion to appropriately manage the disease. Key words: Behcet Disease, HLA B5, Aphthous ulcer, Oral steroids

Behcet disease (BD) is a chronic inflammatory disorder of unknown etiology with a clinical spectrum that has greatly expanded since it was first described in 1937 by Hulusi Behcet as a triple complex of recurrent oral and genital ulcers and uveitis [1]. Although BD has a worldwide distribution, it is mainly seen in Far East and Middle East countries [2,3]. The highest prevalence is reported from Turkey, 80–370 per 100 000, and ranges from 2 to 30 cases per 100 000 in other Asian countries (16.7 per 100 000 in the Islamic Republic of Iran) [2,3]. The age of disease onset is usually in the second or third decade of life and the male to female ratio is reported to be almost equal. However, the disease runs a more severe course in men and in those with onset before 25 years of age [2]. Since BD does not have pathognomonic symptoms or laboratory findings; diagnosis is made on the basis of the criteria proposed by the International Study Group for Behcet disease in 1990 [4]. According to the criteria, recurrent oral ulceration must be present along with at least 2 of the following: recurrent genital ulceration, eye lesions, skin lesions, a positive pathergy test [2,4,5]. Many disorders, such as cutaneous small vessel vasculitis, inflammatory eye disease, neurological disease, vascular disease, arthritis, are associated with the presence of BD [6]. Pathological features such as smallor large-vessel vasculitis lead to occlusion of the vessels or aneurysm formation [7]. Differential diagnosis of Behcet-induced vasculitis has been described by Kreuger, Hoffman and Merton [8]. More than 200 cases of BD with pulmonary involvement have been reported in the literature. The pulmonary arteries are the second most common site of arterial involvement after the aorta. Aneurysms are more common than thrombosis [2]. Aneurysms associated with BD tend to be multiple and pulmonary artery aneurysms are relatively common, with an associated rate of 1%–10% of patients with BD [9]. Haemoptysis of varying degrees (up to 500 mL) is the most common and predominant symptom [2]. Medical treatment is more beneficial when given in the early stage of the disease [10]. Surgical repair of pulmonary artery aneurysm in BD carries a high risk, with high rates of morbidity and mortality [11]. Recently, endovascular management has been attempted for treatment of vascular involvement. However, when aneurysms are multiple, endovascular treatment is still difficult and challenging. To our knowledge, there is no report on a patient with 4 or more arterial aneurysms treated solely by endovascular management [12,13].

BackgroundBehçet Disease (BD) is rarely reported in children. It a systemic inflammatory condition characterised by autoinflammatory and vasculitic clinical features including recurrent oral aphthosis, genital ulceration, skin, eye, neurological and...

SAT0251 SEX- AND AGE-RELATED DIFFERENCES IN CLINICAL MANIFESTATIONS OF BEHÇET’S DISEASE IN A LARGE COHORT OF CHINA PATIENTS

Background: Behcet's disease (BD) is a complex, multisystem inflammatory disorder characterized by recurrent oral and genital ulcers, uveitis, and systemic involvement [1]. This case highlights the diagnostic challenge in a young female with recurrent mucocutaneous and systemic symptoms following COVID-19 infection which can be yet another way for post COVID-19 immune response to mimic known autoimmune conditions. Case Presentation: A 21-year-old female with no significant past medical history presented with anterior uveitis, recurrent painful oral and genital ulcers, and joint pains following two weeks of COVID-19 infection. Initial workup ruled out multiple viral causes (HIV, syphilis, herpes, CMV, EBV, TB) and revealed normal ACE and lysozyme levels as well as normal ANA and negative HLA B27. She responded to steroids and colchicine, raising the suspicion of Behcet's disease. At her follow-up visit, the patient reported complete resolution of her oral and genital ulcers, uveitis, and joint pains. However, she developed an erythematous, itchy, non-painful rash on her thighs and chest, along with canker sores in her mouth. The patient remains off medications and reports no current symptoms aside from the resolved rash and ulcers. Management and Outcome: Given the resolution of her symptoms, the plan is to continue monitoring without further treatment. Despite meeting some criteria for Behcet's disease, the possibility of post-acute COVID syndrome as the underlying cause is also being considered. If symptoms fully resolve without recurrence, the case may be attributed to COVID-19-related inflammatory sequelae rather than a chronic autoimmune condition. Conclusion: This case underscores the diagnostic complexity of Behcet's disease and highlights the potential role of COVID-19 as a trigger for inflammatory symptoms which was reported with different autoimmune conditions before [2]. Yet, there has been no reports of BD like presentation post COVID-19. Ongoing monitoring is essential to differentiate between self-limited post-COVID inflammatory syndromes and chronic autoimmune diseases like Behcet's. Early identification and individualized management can improve patient outcomes.[1] Nair JR, Moots RJ. Behcet's disease. Clin Med (Lond). 2017 Feb;17(1):71-77. doi: 10.7861/clinmedicine.17-1-71. PMID: 28148585; PMCID: PMC6297594.[2] Hileman CO, Malakooti SK, Patil N, Singer NG, McComsey GA. New-onset autoimmune disease after COVID-19. Front Immunol. 2024 Feb 8;15:1337406. doi: 10.3389/fimmu.2024.1337406. PMID: 38390319; PMCID: PMC10883027.

Assessment of venous wall thickness with Doppler ultrasonography may be a diagnostic tool for Behçet's disease.

Dear Editor: Degos disease (DD) is a rare, thrombo-occlusive vasculopathy that primarily affect the skin, gastrointestinal tract, and central nervous system1. The cutaneous lesions present as characteristic papules with porcelain-white central atrophy and an erythematous raised border. Behcet's disease (BD) is a systemic vasculitis characterized clinically by recurrent oral aphthous and genital ulcers, cutaneous lesions, and iridocyclitis/posterior uveitis2. DD can be found as an apparent idiopathic disease or as a surrogate clinical finding in some connective tissue diseases. Here, we report a patient with cutaneous manifestation of DD, associated with mucocutaneous and systemic BD lesions. A 45-year-old woman had asymptomatic erythematous papules with central, porcelain-white colored umbilication on the trunk and both extremities for 20 years, and some of the lesions had healed to leave atrophic scars (Fig. 1A, B). She also had recurrent oral and genital ulcers for 20 years (Fig. 1C). She had been treated with prednisolone, dapsone, and colchicine for 2 years, but the cutaneous lesions did not improve with BD treatments. Fig. 1 (A, B) Erythematous to brownish papules with porcelainwhite central atrophy. (C) Recurrent oral aphthous ulcer. Histopathologic examination of a typical lesion showed a cell-poor, wedge-shaped area of dermal necrosis with vessel occlusion as well as dermal edema. Occluded vessels with thrombosis and vascular endothelial cell swelling were observed at the base of the wedge-shaped lesion, and the area was surrounded by lymphocytic infiltration (Fig. 2). Copious mucin deposition was visible in the dermis, which also showed positive staining for Alcian blue (data not shown). The laboratory findings for antinuclear antibody, lupus anticoagulant, anticardiolipin antibodies, and antiphospholipid antibodies were all negative. The results of the pathergy test were positive. The patient also had non-erosive asymmetric arthritis, and a 99m Tc-HDP 3-phase bone scan showed hot uptake. She had a scarring change on the retina due to recurrent chorioretinitis. Colonoscopy showed no specific findings for DD or BD. The patient was diagnosed with DD associated with BD. The cutaneous lesions, which were not responsive to BD treatment, improved after 4 months of treatment with aspirin and dipyridamole. Fig. 2 Histopathologic finding showing a wedge-shaped dermal infarct with a broad base toward the epidermis. An occluded dermal vessel is visible at the base of the dermal infarct area, and a lymphohistiocytic inflammatory infiltrate is visible at the lateral ... While the etiology of DD is unknown, evidence suggests a primary vaso-occlusive process. Vascular coagulopathy, endothelial cell damage, and an autoimmune process are considered the major pathogenic mechanisms3,4. In BD, an immune-mediated occlusive vasculitis is found in most sites of the active disease, and the endothelium appears to be the primary target5. The cutaneous lesions of the present patient were characteristic of DD and did not respond to BD treatments. This indicates that, while DD can be associated with BD, it is a distinct disease entity to BD. To the best of our knowledge, although Sweet's syndrome and pyoderma gangrenosum in BD have been reported in association with BD, our patient represents the first reported case of DD associated with BD. With the underlying vaso-occlusive process of both DD and BD, it is reasonable for an association between DD and BD to exist. Whether this patient had the same underlying mechanisms or the findings were coincidental is yet to be determined. We suggest that DD and BD are distinct disease entities but that DD can be associated with BD.

Behcet's Disease with Renal Amyloidosis: Report of a Case from Jordan with Review of the Literature

SAT0530 Clinical features association with hla-b allelic types (B27, B51) in korean patients of behcet’s disease

Background : Behcet's disease is a rare immune mediated small vessel systemic vasculitis in which diagnosis is mainly clinical and requires the presence of recurrent oral ulcers and at least two additional criteria that includes recurrent mouth and genital ulcers, skin lesions, eye inflammation (uveitis) and a positive pathergy reaction. Methods : The case records of an 82 year old female Nigerian who presented to the medical outpatient clinic of the Federal Medical Centre, Yenagoa with a six months' history of recurrent oral ulcers, diarrhoea, arthralgia, blurring of vision, breathlessness, abdominal pain and extreme weakness was reviewed. Results : She responded well to oral corticosteroids. Conclusion : Behcet's disease should be given consideration in the differential diagnosis of pleural effusion in the presence of recurrent mucosal ulcers. Keywords : Behcet’s disease, Pleural effusion, uveitis, vasculitis

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Objective To explore the characteristics of diagnosis and treatment of Behcet's disease (BD) with meningeal thickening and to improve doctors' awareness of such diseases. Methods We reported a missed diagnosis of a patient with BD complicated with diffuse meningeal thickening, and reviewed the related literature. Results A 25-year-old young man, manifested mainly as recurrent headache, fever, recurrent oral ulcer, erythema skin nodules and folliculitis, and his cranial radiology revealed diffuse meningeal thickening and intracranial venous sinus thrombosis. We diagnosed him as neuropathy associated with BD. After the treatment with steroids, cyclophosphamide, infliximab and anti-coagulants, his symptoms improved rapidly. Conclusion In clinical, BD complicated with meningeal thickening is rare, which is easily misdiagnosed or miss-diagnosed. For the patients with unexplained meningeal thickening, the symptoms of BD, such as recurrent oral or genital ulcers, ophthalmitis and skin lesions, should be acquired in detail. In addition to steroids and immunosuppressive agents, anti tumor necrosis factor (TNF)-alpha can also be used in the treat-ment of BD with meningeal thickening. Key words: Behcet's disease; Meningeal thickening; Intracranial venous sinus thrombosis; Infliximab

Background:Behcet’s disease (BD) is a systemic vasculitis of unknown etiology, characterized by recurrent oral and genital erosions and ulcerations, as well as involvement of joints, ocular, vascular, gastrointestinal (GI), and...