scholarly journals Pulmonary Nodules as an Initial Manifestation of Behçet’s Disease

2014 ◽  
Vol 2014 ◽  
pp. 1-3
Author(s):  
M. Malekmohammad ◽  
A. Emamifar
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Artery Aneurysm ◽  
Skin Lesions ◽  
Vascular Involvement ◽  
Recurrent Fever ◽  
Initial Symptom ◽  
Initial Manifestation ◽  
Behcet's Disease

Behçet’s disease (BD) is a systemic vasculopathy, characterized by recurrent oral aphthae, genital ulcers, uveitis, and skin lesions. Although vascular involvement, including venous and arteries of any size, is a usual manifestation, cases with pulmonary thrombosis as the initial symptom are not common in the absence of pulmonary artery aneurysm (PAA). This report describes a 36-year-old man with recurrent fever, nonmassive hemoptysis, and persistent cough with lung nodules in CT scan who had undergone open lung biopsy. On the basis of morphological findings, BD was suggested and more precise evaluation confirmed the diagnosis.

THU0599 PULMONARY ARTERY ANEURYSM, BUDD CHIARI SYNDROME, INTRACARDIAC AND INFERIOR VENA CAVA THROMBOSES: AN UNUSUAL CASE OF BEHÇET’S DISEASE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 541-542
Author(s):  
S. Ousalem ◽  
S. Beaudoin
Keyword(s):  
Pulmonary Artery ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Vena Cava ◽  
Artery Aneurysm ◽  
Vascular Involvement ◽  
Budd Chiari Syndrome ◽  
Behcet's Disease ◽  
Chiari Syndrome

Background:Behçet’s disease (BD) or “Silk Road” disease is a rare multisystemic inflammatory disease of unknown etiology.Vascular involvement manifested as thrombosis, arterial aneurysm, and occlusion can carry a high mortality risk. BD can be a diagnostic conundrum with its broad array of clinical presentations.Objectives:Identifying vasculo-Behçet’s disease and its management.Methods:A 25-year-old man born in Malaysia and known for cirrhosis due to idiopathic Budd Chiari syndrome presented to the emergency room with a transient ischemic attack. An inferior vena cava (IVC) occlusive thrombus and a patent foramen ovale (PFO) were discovered. Thrombolysis, angioplasty, PFO closure, and a transjugular intrahepatic portosystemic shunt (TIPS) procedure were performed. The following year, the patient experienced numerous IVC and TIPS-associated thromboses as well as a right atrial thrombus attached to his PFO closure device, all of which were refractory to anticoagulation. A few months later, the patient suffered from an acute right anterior cerebral artery stroke, with no etiology uncovered at the time. It was later determined that the patient had experienced years of recurrent oral and genital aphthae, thereby prompting a strong clinical suspicion of BD. Six months later, after only one appointment at the rheumatology clinic during which he was prescribed colchicine, the patient presented to the hospital with hemoptysis. A computed tomography (CT) pulmonary angiogram revealed a right lower lobar pulmonary arterial aneurysm with a peripheral thrombus, a right bronchial artery dilatation, and pulmonary emboli. The patient declined anticoagulation and was sent home. Two months later, he returned to the hospital, this time with hematemesis. A repeat CT pulmonary angiogram was performed and showed an increasing pulmonary emboli burden and an enlarging aneurysm. A thrombophilia workup was negative.Results:A diagnosis of BD with pulmonary aneurysms was made and treatment was initiated with methylprednisolone pulses and monthly intravenous cyclophosphamide as recommended by the European League Against Rheumatism. A month later, there was radiological evidence of significant improvement in the burden of pulmonary emboli, an interval decrease in the aneurysm’s diameter, and resolution of the right atrial thrombus.Conclusion:BD with vascular involvement or vasculo-Behçet’s disease can affect small, medium, and large vessels of both the venous and arterial vasculatures and is thought to originate from vessel wall inflammation.Thrombi in vasculo-Behçet’s disease are typically quite adherent to the vessel walls and tend not to embolize. In this case, pulmonary arterial thrombosis burden was significantly decreased after immunosuppression alone, favoring a diagnosis of in situ thrombosis rather then thromboembolism. Moreover, pulmonary artery aneurysm, Budd-Chiari syndrome, and vena cava thrombosis, which are quite uncommon and carry the highest mortality risk in vasculo-Behçet’s, were all present in this case. Early recognition can be life-saving as immunosuppression is the first-line therapy rather than anticoagulation, which carries a significant risk of pulmonary hemorrhage in the presence of a pulmonary artery aneurysm.References:[1]Seyahi, E., Behcet’s disease: How to diagnose and treat vascular involvement. Best Pract Res Clin Rheumatol, 2016. 30(2): p. 279-295.[2]Hamuryudan, V., et al., Pulmonary artery aneurysms in Behcet syndrome. Am J Med, 2004. 117(11): p. 867-70.[3]Kobayashi, M., et al., Neutrophil and endothelial cell activation in the vasa vasorum in vasculo-Behcet disease. Histopathology, 2000. 36(4): p. 362-71.[4]Seyahi, E. and S. Yurdakul, Behcet’s Syndrome and Thrombosis. Mediterr J Hematol Infect Dis, 2011. 3(1): p. e2011026.[5]Hatemi, G., et al., 2018 update of the EULAR recommendations for the management of Behcet’s syndrome. Ann Rheum Dis, 2018. 77(6): p. 808-818Disclosure of Interests:None declared

scholarly journals A Rare Case of Vasculitis Patched Necrosis of Cecum due to Behçet’s Disease

2017 ◽  
Vol 2017 ◽  
pp. 1-3 ◽  
Author(s):  
Ehsan Shahverdi ◽  
Mehdi Morshedi ◽  
Faraneh Oraei-Abbasian ◽  
Maryam Allahverdi Khani ◽  
Roya Khodayarnejad
Keyword(s):  
Behçet’S Disease ◽  
Ischemic Colitis ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Skin Lesions ◽  
Clinical Findings ◽  
Vascular Involvement ◽  
Blurred Vision ◽  
Behcet's Disease ◽  
Autoimmune Condition

Isolated cecal necrosis is a rare form of acute ischemic colitis and a rare cause of surgical abdomen. Behçet’s disease is a multisystemic autoimmune condition which can induce vasculitis. This can result in cecal necrosis while disease involves colon vessels. We describe a patient with complicated Behçet’s disease and ischemic colitis admitted to our hospital. Patient was a 62-year-old female with more accompanying diseases. Histopathologic findings confirmed the diagnosis of ischemic colitis and regarding patient’s vision problem and skin lesions, rheumatologic tests were performed which were positive for HLAB5 and HLAB51 suggestive of Behçet’s disease; the patient was improved with surgery. Abdominal pain can indicate a disease with vascular involvement like Behçet’s disease, especially in the presence of other clinical findings suggestive of the disease including blurred vision and skin lesions. An abdominal computerized tomography (CT) scan is very diagnostic in the same patients.

scholarly journals Gender Differences in Behçet’s Disease Associated Uveitis

2014 ◽  
Vol 2014 ◽  
pp. 1-8 ◽  
Author(s):  
Didar Ucar-Comlekoglu ◽  
Austin Fox ◽  
H. Nida Sen
Keyword(s):  
Gender Differences ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Vascular Involvement ◽  
Unknown Etiology ◽  
Behcet's Disease ◽  
Male Patients

Behçet’s disease is a systemic vasculitis of unknown etiology, characterized by oral and genital ulceration, skin lesions, and uveitis as well as vascular, central nervous system, and gastrointestinal system involvement. It is prevalent in the Middle East, Mediterranean, and Eastern Asia. The aim of this review is to evaluate the gender differences in clinical manifestations of Behçet’s disease, treatment responses, mortality, and morbidity. Behçet’s disease has been reported to be more prevalent in males from certain geographic regions and particular ethnic groups; however, recent reports indicate more even gender distribution across the world. There are gender differences in clinical manifestations and severity of the disease. Ocular manifestations, vascular involvement, and neurologic symptoms are more frequently reported in male patients whereas oral and genital ulcers, skin lesions, and arthritis occur more frequently in female patients. The disease can have a more severe course in males, and overall mortality rate is significantly higher among young male patients.

Infliximab treatment in refractory vascular Behcet’s disease: A single-center experience

Vascular ◽  
2020 ◽  
Vol 28 (6) ◽  
pp. 829-833
Author(s):  
Demet Yalçın Kehribar ◽  
Metin Ozgen
Keyword(s):  
Behçet’S Disease ◽  
Oral Anticoagulant ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Vascular Involvement ◽  
Infliximab Treatment ◽  
Single Center ◽  
Vascular Events ◽  
Behcet's Disease ◽  
Significant Difference

Objective This study aims to investigate the efficacy and reliability of infliximab treatment in Behcet’s disease with vascular involvement. Methods This single-center retrospective study included a total of 18 patients diagnosed with Behcet’s disease with vascular involvement who were initiated infliximab treatment after exhibiting resistance to conventional immunosuppressive treatments. Results Seventeen patients achieved remission with infliximab treatment. While 18 patients were receiving a median of 50 (IQR: 20–61) mg/day equivalent of methylprednisolone before infliximab treatment, after infliximab treatment, only four patients were receiving 4 mg/day equivalent of methylprednisolone ( p < 0.001). Only 4 patients were receiving oral anticoagulant treatment during infliximab treatment, and compared to the patients who were not receiving oral anticoagulants, there was no significant difference between the two groups according to occurrence of new vascular events. Conclusion Infliximab seems to be an effective and reliable treatment in Behcet’s disease with vascular involvement and may also allow reduced dosage or even the discontinuation of corticosteroids. The results of our study suggest that oral anticoagulant use is unnecessary in Behcet’s disease with vascular involvement. However, further long-term randomized controlled studies are needed to investigate the length of infliximab regimen, whether or not it should be discontinued, and if so, whether or not immunosuppressants should be given as maintenance after discontinuation.

scholarly journals Behcet's disease with major vascular involvement

2013 ◽  
Vol 2013 (nov08 1) ◽  
pp. bcr2013200893-bcr2013200893 ◽  
Author(s):  
L. N. Geng ◽  
D. Conway ◽  
S. Barnhart ◽  
J. Nowatzky
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Vascular Involvement ◽  
Behcet's Disease

scholarly journals Immunopathogenesis of Ocular Behçet’s Disease

2014 ◽  
Vol 2014 ◽  
pp. 1-13 ◽  
Author(s):  
Un Chul Park ◽  
Tae Wan Kim ◽  
Hyeong Gon Yu
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Retinal Vasculitis ◽  
Skin Lesions ◽  
Unknown Etiology ◽  
Ocular Involvement ◽  
Inflammatory Disorder ◽  
General Hypothesis ◽  
Behcet's Disease

Behçet’s disease (BD) is a chronic recurrent systemic inflammatory disorder of unknown etiology characterized by oral and genital ulcerations, skin lesions, and uveitis. The ocular involvement of BD, or Behçet’s uveitis (BU), is characterized by panuveitis or posterior uveitis with occlusive retinal vasculitis and tends to be more recurrent and sight threatening than other endogenous autoimmune uveitides, despite aggressive immunosuppression. Although pathogenesis of BD is unclear, researches have revealed that immunological aberrations may be the cornerstone of BD development. General hypothesis of BD pathogenesis is that inflammatory response is initiated by infectious agents or autoantigens in patients with predisposing genetic factors and perpetuated by both innate and acquired immunity. In addition, a network of immune mediators plays a substantial role in the inflammatory cascade. Recently, we found that the immunopathogenesis of BU is distinct from other autoimmune uveitides regarding intraocular effector cell profiles, maturation markers of dendritic cells, and the cytokine/chemokine environment. In addition, accumulating evidence indicates the involvement of Th17 cells in BD and BU. Recent studies on genetics and biologics therapies in refractory BU also support the immunological association with the pathogenesis of BU. In this review, we provide an overview of novel findings regarding the immunopathogenesis of BU.

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