scholarly journals Recurrent Neuroretinitis: A Unique Presentation of Behçet’s Disease in a Child

2020 ◽  
Vol 11 (3) ◽  
pp. 516-522
Author(s):  
Gilad Rabina ◽  
Gil Amarilyo ◽  
Dinah Zur ◽  
Liora Harel ◽  
Zohar Habot-Wilner
Keyword(s):  
Visual Acuity ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Abdominal Ultrasound ◽  
Intermediate Uveitis ◽  
Behcet's Disease ◽  
Protein Levels ◽  
First Case ◽  
Finger Counting

We describe a case of Behçet’s disease (BD) in a young child that presented with recurrent neuroretinitis and developed retinal lesions during follow-up. A 4.5-year-old girl presented with fever of 39.5°C, erythema nodosum in her legs, bilateral knee arthritis, and perineum aphthae. On ocular examination, visual acuity was 20/25 in both eyes. Right eye examination was normal and the left eye (LE) showed mild anterior and intermediate uveitis, normal optic disc, and a macular star appearance. Laboratory workup demonstrated elevated C-reactive protein levels, a normal abdominal ultrasound, and a normal colonoscopy. The patient was diagnosed with BD. One month post initial presentation, the patient presented with visual acuity of finger counting in the LE with significant anterior uveitis, mild intermediate uveitis, and recurrent neuroretinitis. Under treatment of IV methylprednisolone, oral betamethasone, infliximab, and colchicine, a complete systemic remission was noticed, and uveitis became quiescent. On last examination, 4.5 years post first presentation, visual acuity was 20/25 in both eyes and the LE demonstrated a remnant of a juxtafoveal retinal scar. To the best of our knowledge, this is the first case of neuroretinitis presenting as a manifestation of pediatric BD. Ophthalmologists should be aware of these unique manifestations of ocular BD.

Neuro-ophthalmological manifestations of Behçet’s disease

2018 ◽  
Vol 103 (1) ◽  
pp. 83-87 ◽  
Author(s):  
Ammr Alghamdi ◽  
Bahram Bodaghi ◽  
Chloé Comarmond ◽  
Anne Claire Desbois ◽  
Fanny Domont ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Behçet’S Disease ◽  
Cranial Nerve ◽  
Cranial Nerve Palsy ◽  
Nerve Palsy ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Visual Outcome ◽  
Ophthalmological Examination ◽  
Behcet's Disease

BackgroundThe neuro-ophthalmological manifestations of Behcet’s disease (BD) are rare, and data regarding their characteristics and outcome are lacking.ObjectiveTo report prevalence, main characteristics and outcome of neuro-ophthalmological manifestations in BD.Patients and methodsThis is a retrospective monocentric study of 217 patients diagnosed with neuro-Behçet’s disease (NBD), of whom 29 (13.3%) patients presented with neuro-ophthalmological manifestations (55% of men and mean±SD age of 26±8 years). All patients underwent a detailed ophthalmological examination and were followed up in the internal medicine and the ophthalmology departments.ResultsNeuro-ophthalmological manifestations were the first presentation of BD in 45% of patients and developed later in the course of the disease in 55% of patients. They are divided into parenchymal (PM) and non-parenchymal (NPM)-related manifestations in, respectively, 13 patients (45%) and 16 patients (55%). PM included papillitis in seven patients (53.8%), retrobulbar optic neuritis in four patients (30.8%) and third cranial nerve palsy in two patients (15.4%). NPM included papilloedema related to cerebral venous thrombosis in all 16 patients, of whom 6 patients (37.5%) had sixth cranial nerve palsy. At initial examination, 93.1% of patients had visual alterations, including mainly decreased visual acuity visual field defects and/or diplopia. All patients were treated with corticosteroids and 79% of patients received immunosuppressive agents. After treatment, the visual outcome improved or stabilised in 66.7% of patients while it worsened in 33.3. The mean±SD logarithm of the minimum angle of resolution visual acuity improved from 0.4±0.3 at diagnosis to 0.2±0.3 after therapy. 10.3% and 3.4% of patients were legally blind at diagnosis and after therapy, respectively.ConclusionNeuro-ophthalmological manifestations of BD represented 13% of NBD. They could be potentially severe and disabling. Prompt treatment is the key factor in improving visual outcome.

The First Case of Multifocal Osteonecrosis in Behcet’s Disease

2020 ◽  
Author(s):  
Seyedeh Tahereh Faezi ◽  
Mohammad Nejadhosseinian ◽  
Farhad Shahram ◽  
Nahid Sadighi ◽  
Masoumeh Banihashemian ◽  
...  
Keyword(s):  
Shoulder Pain ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Oral Treatment ◽  
Retinal Vasculitis ◽  
Anticardiolipin Antibody ◽  
Immunoglobulin M ◽  
Behcet's Disease ◽  
First Case

Background: Osteonecrosis is death of bone tissue due to interruption of the blood supply. It can occur in autoimmune disease due to multiple mechanisms. It occurs rarely in some kind of multisystem disease such as Behcet’s disease (BD). The aim of this article is to present a case of BD with multifocal osteonecrosis. Case Presentation: A 26-year-old woman with oral aphthosis, panuveitis, and retinal vasculitis was diagnosed to have BD, and oral treatment of prednisolone (0.5 mg/kg/day) together with azathioprine (2 mg/kg/day) and intravenous (IV) cyclophosphamide (CYC) (0.75 g/m2) was administered for her. Prednisolone was tapered to 15 mg/day after 3 months. Because of unresponsiveness of eye involvement to traditional immunosuppressive therapy, infliximab (5 mg/kg) was started for her and IV CYC was discontinued. 4 months after initial therapy, she complained of her right shoulder pain. Regarding to shoulder pain, magnetic resonance imaging (MRI) showed osteonecrosis of right shoulder. After diagnosis of osteonecrosis, prednisolone was discontinued by herself. 3 months later, she complained of both knees, both hips, and right ankle pain. According to previous diagnosis of osteonecrosis of her right shoulder, MRI was done for all involved joints, which revealed osteonecrosis of all of them. After diagnosis of multifocal osteonecrosis, the following tests were done that all results were normal: anticardiolipin antibody (ACA) [immunoglobulin G (IgG), immunoglobulin M (IgM)], lupus anticoagulant (LA), anti-beta-2 glycoprotein 1 (antiB2GP1) (IgG, IgM), coagulation tests, and lipid profiles. Conclusions: Bone infarction should be considered in differential diagnosis of patients with joint pain and without the evidence of arthritis; however, it rarely occurs in patients with BD.

scholarly journals Efficacy of TNF-α antagonist and other immunomodulators in the treatment of patients with ophthalmologic manifestations of Behcet's disease and HLA B51 positive vasculitis

2012 ◽  
Vol 69 (2) ◽  
pp. 168-174 ◽  
Author(s):  
Gordana Zlatanovic ◽  
Svetlana Jovanovic ◽  
Dragan Veselinovic ◽  
Maja Zivkovic
Keyword(s):  
Visual Acuity ◽  
Disease Activity ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Unknown Etiology ◽  
Behcet's Disease ◽  
Therapeutic Method ◽  
Tnf Antagonist ◽  
Pathergy Test

Bacground/Aim. Behcet's disease is genetically conditioned, immune-mediated multisystem occlusive vasculitis of small blood vessels, espesially venules, of unknown etiology. The aim of this study was to analyze the clinical features, disease activity and therapy of the patients with ophthalmologic manifestation of Behcet's disease. Methods. In this study symptoms and signs of the disease were analyzed both prospectively and retrospectively during the active manifestation of the disease. The diagnosis was reached according to the International Criteria for Behcet's Disease (2006). The treatment effects were evaluated based on the presence of the best corrected visual acuity and the inflammation of the vitreous humour before and after the application of our therapeutic method. The applied therapeutic modality consisted of the primary application of corticosteroid therapy in the active stage of the disease complemented with the choice of drugs from the immunosuppressive group. In this study there drugs were cyclosporine or methotrexate. A treatment refractory patients with poor vision prognosis were treated with a third drug, the biological preparation infliximab, a tumor necrosis factor-alpha (TNF-?) antagonist. Results. The mean age of 11 patients with ophthalmologic manifestation of Behcet's disease was 50.6 years. HLA B-5(51) was positive in 81% of the patients while 36% of the patients had positive pathergy test. Changes in affected eyes included vitritis (100%), posterior uveitis (45%), panuveitis (54%), retinal vasculitis (54%), cystoid macular edema (54%), and cystoid degeneration (18%). Increased intraocular pressure was observed in 27% of the patients. There was no statistically significant variation in disease activity parameters in any of the patients (p > 0.05). A statistically significant improvement in visual acuity (p < 0.05) and a high statistically significant decrease of inflammation of the worst affected eyes (p = 0.001) were detected. Conclusion. Our therapeutic method is useful for producing the optimal therapeutic plan for the acute - chronic stage of the difficult ophtamological manifestation of Behcet's desease as well as the prevention of relapse. However the high cost of the therapy and the potential complications should be taken into consideration when prescribing this therapy, especially a TNF-? antagonist.

scholarly journals Decreased MicroRNA-155 in Behcet’s disease leads to defective control of autophagy thereby stimulating excessive proinflammatory cytokine production

2020 ◽  
Author(s):  
Liang Liang ◽  
Qingyun Zhou ◽  
Lujia Feng ◽  
Aize Kijlstra ◽  
Peizeng Yang
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Cytokine Production ◽  
Behçet's Disease ◽  
Beclin 1 ◽  
Mtor Signaling Pathway ◽  
Behcet's Disease ◽  
Protein Levels ◽  
Transmission Electron ◽  
Tnf Α

Abstract Background: Earlier we reported that the microRNA (miR)-155 expression in dendritic cells (DCs) from Behcet’s disease (BD) patients was decreased and affected cytokine production of DCs. In this study, we investigated the mechanisms whereby miR-155 regulates cytokine production by DCs.Methods: The formation of autophagosomes in DCs was detected by transmission electron microscopy. Western blotting was used to detect the protein levels of LC3, Beclin-1, P62, p-mTOR and p-Akt in DCs. TNF-α, IL-6 and IL-1β expression were investigated by ELISA. MiR-155 mimics were transfected to DCs to evaluate its effects on autophagy and cytokine production. RNA interference was used to downregulate the expression of TAB2.Results: The formation of autophagosomes was found in DCs of active BD patients. The expressions of LC3-Ⅱ, Beclin-1, P62, IL-6, IL-1β and TNF-α were significantly increased in DCs of active BD patients compared to that of healthy controls. The autophagy promoter (3-MA) and inhibitor (rapamycin) significantly decreased or increased the expression of TNF-α, IL-6 and IL-1β by DCs. The expression of LC3-Ⅱand Beclin-1 were significantly increased, but the expression of P62 proteins was decreased in DCs transfected with miR-155 mimics or after TAB2 was downregulated. The expression of TNF-α, IL-6 and IL-1β were decreased in DCs after miR-155 was upregulated or TAB2 was downregulated. The ratios of p-Akt/Akt and p-mTOR/mTOR were decreased in DCs after miR-155 was upregulated.Conclusions: These results suggest that miR-155 affects the production of TNF-α, IL-6 and IL-1β by DCs through activation of the Akt/mTOR signaling pathway and by affecting the process of autophagy.

scholarly journals Secondary hand infection with Eikenella corrodens and Staphylococcus aureus in a patient with Behcet's disease: a case report

2022 ◽  
Vol 50 (1) ◽  
pp. 030006052110727
Author(s):  
Yaoting Liu ◽  
Jiayun Zhong ◽  
Haiqing Hu ◽  
Qing Hou ◽  
Xianfeng Chen ◽  
...  
Keyword(s):  
Staphylococcus Aureus ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Eikenella Corrodens ◽  
Behcet's Disease ◽  
First Case ◽  
Immunosuppressed Patients ◽  
History Of ◽  
And Staphylococcus Aureus

We report the first case of a woman with Behcet's disease (BD) with multiple hand ulcers secondary to coninfection by Eikenella corrodens and Staphylococcus aureus resulting in necrotizing fasciitis. She had a long history of BD including long courses of prednisone and immunosuppressants. The patient was hospitalized for multiple superficial ulcers, swelling, and infection of the hands. After admission, pus culture examination revealed rare coinfection by E. corrodens and S. aureus. We administered moxifloxacin and vancomycin to control infection and methylprednisolone to control BD. We performed incision, drainage, and debridement of the ulcer surface on the hands to reduce the pus on the wound surface. E. corrodens infections occur in immunosuppressed patients and contribute to coinfections, particularly in patients with BD in whom destruction of the skin immune barrier increases risk to secondary infections. For severe and complicated hand infections, efforts should be made to identify pathogenic microorganisms so appropriate antibiotics and other interventions can be given to control the infection.

scholarly journals Successful Treatment and Secondary Prevention of Venous Thrombosis Secondary to Behçet Disease with Rivaroxaban

2016 ◽  
Vol 2016 ◽  
pp. 1-3
Author(s):  
Ana Boban ◽  
Catherine Lambert ◽  
Cedric Hermans
Keyword(s):  
Adverse Effect ◽  
Secondary Prevention ◽  
Venous Thrombosis ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Secondary Prophylaxis ◽  
Behcet's Disease ◽  
First Case ◽  
Anticoagulant Drug

We here present the successful initial treatment and secondary prophylaxis of superficial venous thrombosis secondary to Behçet’s disease by a novel anticoagulant drug, rivaroxaban (Xarelto®). To our knowledge, this is the first case of using an oral direct inhibitor of FXa in this setting. Our findings are promising; the outcome was favourable without any adverse effect noted. We propose that the patients with Behçet’s disease and venous thrombosis might benefit from the advantages of the new anticoagulant drug.

Immune keratitis: An unusual primary presentation of neuro-Behçet’s disease

2018 ◽  
Vol 29 (4) ◽  
pp. NP5-NP8
Author(s):  
Jitender Jinagal ◽  
Aniruddha Agarwal ◽  
Avinash Negi ◽  
Gaurav Gupta ◽  
Vikas Sharma ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Disease Patient ◽  
Behcet's Disease ◽  
Ocular Discomfort ◽  
Laboratory Investigations ◽  
Systemic Examination ◽  
The Right

A 19-year-old Nepalese male presented with complaints of bilateral ocular discomfort, photophobia, watering, and redness for 1 week. Visual acuity was 6/12 and 6/60 in the right and left eye, respectively. On biomicroscopic examination, presence of peripheral stromal infiltrates with conjunctival follicles was noted; infiltrates progressed to involve central cornea with further decrease in vision over next few days. After ruling out infectious keratitis, detailed systemic examination and laboratory investigations were diagnostic of neuro-Behçet’s disease. Patient responded to systemic steroidal and immunosuppressive therapy characterized by corneal healing and visual acuity improvement to 6/6 and 6/9. Although rare, but neuro-Behçet’s disease can primarily present as bilateral immune keratitis and every case of bilateral keratitis needs early systemic evaluation after ruling out infective etiologies.

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