scholarly journals Uveitis-induced Refractory Ocular Hypotony Managed with High-dose Latanoprost

2020 ◽  
Author(s):  
Fariba Ghassemi ◽  
Mohammad Reza Niyousha ◽  
Narges Hassanpoor ◽  
Hassan Khojasteh
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Silicone Oil ◽  
Behçet's Disease ◽  
Treatment Modalities ◽  
High Dose ◽  
Behcet's Disease ◽  
Multiple Treatment ◽  
Ocular Hypotony ◽  
History Of

Purpose: To report a case of refractory ocular hypotony due to chronic Behcet’s disease with good response to high-dose topical latanoprost. Case Report: We present a 26-year-old man with a known history of Behcet’s disease who developed decreasing vision and severe ocular hypotony that was refractory to multiple treatment modalities including subtenon triamcinolone acetonide, ibopamine, pars plana vitrectomy, and silicone oil injection. We decided to try high-dose topical latanoprost for the management of ocular hypotony based on recent reports. After six months, intraocular pressure (IOP) increased by 5 mm Hg, became stable at 7 mm Hg, and remained unchanged at month 24. Conclusion: High-dose topical latanoprost could lead to significant increase in IOP in uveitis-induced refractory ocular hypotony.

scholarly journals AB0531 GOLIMUMAB IN THE TREATMENT OF SEVERE AND/OR REFRACTORY VASCULO-BEHÇET’S DISEASE: A SINGLE-CENTRE EXPERIENCE IN CHINA

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1562.1-1563
Author(s):  
L. Sun ◽  
J. Liu ◽  
W. Zheng
Keyword(s):  
Aortic Regurgitation ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
High Dose ◽  
Efficacy And Safety ◽  
Behcet's Disease ◽  
Severe Aortic Regurgitation ◽  
Tnf Α

Background:Vascular involvement is one of the leading causes of mortality and morbidity in Behcet’s Disease (BD)1. Surgical treatments are difficult for Vaculo-BD (VBD) patients due to the high risk of serious postoperative complications without effective and promptly perioperative immunotherapy2, 3. Anti-tumor necrosis factor alpha (TNF-α) therapy has been reported as a potential treatment in severe VBD, e.g. infliximab (IFX) and adalimumab (ADA). However, only few case reports are available regarding the fully humanized monoclonal antibody to TNF-α, golimumab (GOL), in the management of VBD4.Objectives:The objective of this study was to report the efficacy and safety of GOL for the treatment of severe and/or refractory VBD.Methods:We retrospectively analyzed the efficacy and safety profile of patients with severe and/or refractory VBD treated with GOL in our medical center between 2018 to 2020.Results:Nine VBD patients (8 male and 1 female) were enrolled, with a mean age and median course of 37±8.6 years and 72 months (range 12 to 300), respectively. Cardiac involvements (severe aortic regurgitation secondary to BD) were presented in 7 patients, including 2 patients with post-operative paravalvular leakage (PVL) after aortic valve replacement surgery. Multiple vascular lesions were documented in the other 2 patients, including one patient with life-threatening multiple pulmonary aneurysms, pulmonary thromboembolism and recurrent deep vein thrombosis, and another patient with abdominal aortic pseudoaneurysm and multiple artery stenosis and occlusion. Prior to GOL therapy, all patients experienced disease progression despite high-dose glucocorticoids combined with multiple immunosuppressants. Moreover, seven patients required effective and fast control of inflammation and a decrease of glucocorticoid dose during the perioperative period. They were treated with GOL, 50mg every 4 weeks, in combination with background low-or medium-dose glucocorticoids and immunosuppressants, for a median of 6 (range 3-15) months. After a mean duration of follow-up of 10 (range 2-6) months, all patients achieved improvement both in clinical symptoms and serum inflammation markers. The ESR level [4.88±4.94 mm/h vs 31.13±31.78mm/h, P<0.01] and CRP level [1.9 (0.11-3.73)mg/L vs 24.3 (0.4-85.57)mg/L, P<0.01] significantly decreased. The dosage of glucocorticoid[10 (0-15) vs 40 (0-100)mg/d, P<0.01] effectively tapered, indicating a potential steroid-sparing effect. No newly-onset aneurysm and recurrent venous thrombosis were observed. Also, one patient had a marked reduction in size and number of pulmonary aneurysms. No post-operative PVL was observed in the five patients after Bentall operation with a median follow-up of 10 months. One patient with severe aortic regurgitation remained stable and without surgical intervention with the treatment of GOL for 16 months. No severe complication occurred in one patient after underwent endovascular repair of abdominal aorta for 8 months. GOL was well-tolerated, and no serious adverse event was observed.Conclusion:Our results suggested that GOL is safe and effective for the treatment of patients with severe and / or refractory VBD. Further controlled studies are warranted to confirm the therapeutic potential of GOL in VBD patients.Disclosure of Interests:None declared

scholarly journals High-Dose Cyclophosphamide Without Stem Cell Rescue for the Treatment of Refractory Behcet's Disease

2011 ◽  
Vol 41 (2) ◽  
pp. 301-304 ◽  
Author(s):  
Charles F. Henderson ◽  
Robert A. Brodsky ◽  
Richard J. Jones ◽  
Stuart M. Levine
Keyword(s):  
Stem Cell ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
High Dose ◽  
Behcet's Disease ◽  
Stem Cell Rescue

475 A rare cause of right ventricular mass

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
A Briosa ◽  
A R Almeida ◽  
A C Gomes ◽  
A R Pereira ◽  
A Marques ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Large Mass ◽  
Behcet's Disease ◽  
Ventricular Mass ◽  
Right Ventricular Mass ◽  
History Of ◽  
The Right

Abstract Introduction Intracardiac masses are always a challenging diagnosis, especially when it involves the right side of the heart. There are multiples etiologies that can be responsible for these masses, namely thrombosis, neoplasm or vegetations. Occasionally, these may be related to an autoimmune process not yet discovered. Case Report 17-year-old male, with a previous history of genital ulcers, medicated with penicillin with complete resolution of symptoms. In January 2019, he started an history of recurrent fever, associated with right anterior thoracalgia, weight loss and oral afthosis. He went to the emergency department several times, where he was medicated with antibiotic, with partial symptom relief. Three months later, he returned to medical attention due to an episode of abundant hemoptysis, followed by hematemesis and cough. At hospital admission, he was hemodynamically stable, tachycardic (100/min) and with occasional episodes of cough. Cardiac and pulmonary auscultation were unremarkable. Thoracic CT revealed the presence of pulmonary thromboembolism (PTE) and a large mass in the right ventricle (RV). It was performed an echocardiogram (echo) that confirmed the presence of a large mass in the RV (50x53mm) from which a projecting hypermobile mass appeared to prolapse into the right atrium. Taking into account the diagnosis of PTE and the presence of a right ventricular mass, the patient was hospitalized and started anticoagulation. The case was immediately discussed with cardiac surgery, that confirmed that there was no surgical indication. During hospitalization, there were no more episodes of hemoptysis or hematemesis.Consecutive echos were performed, that did not reveal a significant decrease in mass dimensions despite anticoagulation. Viral serologies and autoimmunity panel were all negative. Cardiac RMI was performed raising the suspicion of a possible mass covered with thrombus. After discussion with rheumatology, and according to clinical signs, the hypothesis of vasculitis was placed, and the patient started treatment with steroids. This treatment had to be suspended after a few days due to an infectious intercurrence. After a course of antibiotic therapy, the patient started therapy with cyclophosphamide with good clinical and echocardiographic response (reduced mass dimensions). It was admitted Behçet’s disease with cardiac complications, and the patient was referred to the rheumatology consultation. Conclusion Behçet’s disease is a multi-system, chronic disorder that behaves like vasculitis.There are some typical clinical manifestations associated with this disease, such as oral and genital afthosis, uveitis, arthritis, skin lesions and nervous system involvement.Presentations with cardiac symptoms are one of the extremely rare manifestations of this disease, posing a challenge for the treating physician. Abstract 475 Figure. Right ventricular mass

scholarly journals Behcet’s Disease with Intracardiac Thrombus Presenting with Fever of Unknown Etiology

2015 ◽  
Vol 2015 ◽  
pp. 1-3
Author(s):  
Sajal Ajmani ◽  
Durga Prasanna Misra ◽  
Deep Chandh Raja ◽  
Namita Mohindra ◽  
Vikas Agarwal
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Thrombus Formation ◽  
Young Male ◽  
Unknown Etiology ◽  
High Dose ◽  
Intracardiac Thrombus ◽  
High Grade Fever ◽  
Behcet's Disease

A young male was referred to us for evaluation of fever of unknown origin (FUO). He had history of recurrent painful oral ulcers for one year and moderate to high grade fever, pustulopapular rash, and recurrent genital ulcers for 6 months and hemoptysis for 3 days. He was detected to have intracardiac thrombi and pulmonary arterial thrombosis along with underlying Behcet’s disease (BD). Patient responded to high dose prednisolone (1 mg/Kg/day) along with monthly parenteral cyclophosphamide therapy. This case highlights the fact that BD is an important cause for pulmonary artery vasculitis with intracardiac thrombus formation, and such patients can present with FUO.

Recurrent vulval ulceration: could it be Behçet's disease?

2012 ◽  
Vol 23 (9) ◽  
pp. 683-684
Author(s):  
S Patel ◽  
K Prime
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Disease ◽  
Holistic Approach ◽  
Nucleic Acid Amplification ◽  
Behcet's Disease ◽  
Oral Ulcers ◽  
Caribbean Woman ◽  
History Of

This report describes a young black Caribbean woman with a three-year history of recurrent genital ulceration initially diagnosed as genital herpes. This diagnosis had been made clinically by the general practitioner but never confirmed on culture or nucleic acid amplification testing; sequential treatment with aciclovir and famciclovir over a four-month period did not alleviate her genital symptoms. Presentation to our genitourinary (GU) medicine clinic identified painful aphthous genital and oral ulcers, erythema nodosum bilaterally and a history of eye irritation. A clinical diagnosis of Behçet's disease was made. Treatment with hydrocortisone mouth pellets and dermovate ointment was initiated in addition to dermatology and ophthalmology referral. This report reminds clinicians to consider alternative diagnoses in clinical practice when faced with patients who are resistant to treatment. It also highlights the importance of recognizing systemic disease and maintaining a holistic approach when treating patients.

scholarly journals Behçet’s Disease and Tuberculosis: A Complex Relationship

2020 ◽  
Author(s):  
Sara Mendonça Freitas ◽  
Joana Silva Marques ◽  
Ana Grilo ◽  
Rodolfo Gomes ◽  
Fernando Martos Gonçalves
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Gastrointestinal Involvement ◽  
Behcet's Disease ◽  
History Of ◽  
Systemic Manifestations ◽  
Work Up

Behçet’s disease (BD) is a systemic vasculitis characterized by recurrent orogenital ulceration and several systemic manifestations (such as gastrointestinal involvement, vascular disease or arthritis). The pathogenesis is still unknown but the trigger role of certain pathogens such as Mycobacterium tuberculosis is well documented. Furthermore, patients with BD are more susceptible to tuberculosis due to immunity defects. Here, we describe the case of a 70-year-old woman with a history of recurrent oral aphthae and inflammatory arthritis presenting with extensive thrombosis of left upper limb major veins, a positive HLA B51 genotype and colon ulceration; hence, BD diagnosis was made after excluding other causes. Simultaneously, the patient had cutaneous abscesses not associated with immunosuppressive therapy with continuous development, and after recurrent negative tuberculosis work-up, M. tuberculosis was isolated in an abscess culture.

Undiagnosed Behçet's Disease Complicated by Multiple Pseudoaneurysms and COVID-19 Infection

2021 ◽  
Author(s):  
Aanchal Mehta ◽  
Julien Al Shakarchi ◽  
Lewis Meecham ◽  
Santhosh Vijay ◽  
Richard Downing
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Vascular System ◽  
Behçet's Disease ◽  
Sinus Thrombosis ◽  
Periodic Fever ◽  
Lower Lobe ◽  
Prior History ◽  
Behcet's Disease ◽  
History Of

AbstractBehcet's disease (BD) is a rare multiorgan systemic disorder characterized by recurrent episodes of acute inflammation. Involvement of the vascular system, gastrointestinal tract, and central nervous system portends a poor prognosis.We report the case of a 54-year-old man who presented with a 2-week history of symptoms attributable to infrarenal aortic and left tibioperoneal trunk pseudoaneurysms, defined by CT angiography which also revealed right lower lobe pulmonary artery (PA) and right anterior tibial arterial aneurysms. A prior history of recurrent oral ulceration, periodic fever, cerebral venous sinus thrombosis, and aseptic endocarditis with pulmonary emboli invoked a diagnosis of BD. Immunosuppression was commenced immediately, following synchronous endovascular and open arterial intervention, except the PA aneurysm.He developed a fever and cough 8 days postoperatively as a consequence of COVID-19 infection from which he recovered without complications.The management of the patient's four aneurysms in different vascular territories and postoperative COVID-19 infection in the presence of mandatory immunosuppression are discussed.

scholarly journals Behcet’s Disease with Upper GI Bleeding

2020 ◽  
Vol 2020 ◽  
pp. 1-3
Author(s):  
Melaku Getachew ◽  
Nebiyou Seyoum ◽  
Finot Debebe
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Sub Saharan Africa ◽  
Cerebral Vein ◽  
Multisystem Disease ◽  
Behcet's Disease ◽  
Pathergy Test ◽  
History Of ◽  
Sub Saharan

Introduction. Behcet’s disease is a multisystem disease. In sub-Saharan Africa, the prevalence of this disease is not known, with only one case report from Ethiopia. Case Presentation. We describe a case of a 29-year-old Ethiopian male who presented to the emergency room of Tikur Anbessa specialized hospital with 4 days history of back pain, recurrent history of oral and genital ulcers, right eye blindness, chronic cerebral vein thrombosis, gastrointestinal bleeding, aortic aneurysm with dissection, and positive pathergy test. He is retrospectively diagnosed with Behcet’s disease according to both the International Criteria for Behcet’s Disease (ICBD) and the International Study Group (ISG) consensus. Conclusion. Even if Behcet’s disease is rare in sub-Saharan Africa, it is important to know the clinical presentation for timely diagnosis and urgent management.

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