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.

Stellar Neuroretinitis as Presenting Sign of Behcet’s Disease

2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Jouidi W ◽  
◽  
Moutarajji KL ◽  
Abdellah E ◽  
Berraho A ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Serous Retinal Detachment ◽  
Anterior Segment ◽  
Behcet's Disease ◽  
History Of ◽  
Post Traumatic ◽  
The Right

A 40-year-old One-eyed patient (right eye) acuity history of post-traumatic burst in his left eye at the age of 6 years. Consult for a decrease of vision on his right eye that has progressed for 3 weeks. Ophthalmic examination found visual acuity: 2/10 P10 in the right eye, the left eye presents enophthalmos with an opacified cornea; the anterior segment was normal in the right eye, fundus examination shows hyalitis graded 1+, papillary edema, peripapillary hemorrhages and stellar macular exudates, cottony nodules, copper arteries with vascular sheathing of the superior temporal branch and areas of retinal ischemia (Figure 1). The inflammatory and serological assessment is negative. Fluorescein retinal angiography shows papillary diffusion of fluorescein and vasculitis (Figure 2). We find serous retinal detachment on OCT (Figure 3). The general examination found bipolar aphthosis. The diagnosis of Behçet’s disease is retained. Treatment with immunosuppressants allowed the regression of papillary edema with persistence of some macular exudates and great improvingin visual acuity.

Optic nerve perineuritis and retroperitoneal panniculitis: rare first presentations of Behçet’s disease

2021 ◽  
Vol 14 (7) ◽  
pp. e243997
Author(s):  
Katsunobu Yoshioka ◽  
Eiko Morita
Keyword(s):  
Optic Nerve ◽  
Eye Movement ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Leg Pain ◽  
Warning Sign ◽  
Behcet's Disease ◽  
Eye Pain ◽  
The Right

A 46-year-old woman was admitted to our hospital because of high fever and lumbago. CT revealed increase in density of fat tissue around the aorta, suggesting retroperitoneal panniculitis. The following day, she reported of leg pain, pain in the mouth, painful urination and right eye pain that was exacerbated by eye movement. We observed erythema nodosum, aphthous stomatitis and genital ulceration. Ophthalmologic examination revealed mild right optic disc oedema. Visual acuity was normal in both eyes. MRI with gadolinium infusion revealed enhancement around the right optic nerve, suggesting optic nerve perineuritis (ONP). A diagnosis of Behçet’s disease was made. She was treated with prednisolone and colchicine with a favourable response. A warning sign of ONP is eye pain that is exacerbated by eye movement. MRI with gadolinium infusion should be performed immediately in such cases to prevent vision-threatening sequelae.

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

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.

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.

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