Rheumatology
Stephan Strobel, Lewis Spitz, Stephen D. Marks in Great Ormond Street Handbook of Paediatrics, 2019
Autoimmune or autoinflammatory diseases: JIA-particularly the systemic form.JDM.SLE.undifferentiated connective tissue disease.sarcoidosis.Behçet disease.
Autoimmune disorders that can be mistaken for viral illness
Avindra Nath, Joseph R. Berger in Clinical Neurovirology, 2020
The CNS is involved in about 5%–10% of patients with Behçet disease. Behçet disease is a chronic relapsing inflammatory disease of unclear cause. The diagnosis of this disease depends on the presence of recurrent oral ulcers with two of the following: Recurrent genital ulcerations, skin lesions, eye lesions, or hyperactivity of skin to nonspecific physical insult (Patergy test) [50]. Some of these patients have a rhombencephalitis, while others have venous sinus thrombosis, sometimes with seizures [51].
Cardiothoracic and Vascular
Vincent Helyar, Aidan Shaw in The Final FRCR, 2017
Behçet disease is a multi-systemic immune-mediated vasculitis affecting both arteries and veins, more common (by four- to five-fold) in young women. The classic presentation is with a triad of oral ulceration, genital ulceration and ocular inflammation.
Tocilizumab for the Treatment of Behçet Uveitis that Failed Interferon Alpha and Anti-Tumor Necrosis Factor-Alpha Therapy
Published in Ocular Immunology and Inflammation, 2018
Hilal Eser Ozturk, Merih Oray, Ilknur Tugal-Tutkun
Behçet disease is a multisystem inflammatory disorder characterized by recurrent inflammatory attacks affecting the eyes, mucocutaneous tissues, blood vessels, joints, gastrointestinal tract, and the brain. Behçet uveitis (BU) is classically known as bilateral nongranulomatous panuveitis and retinal vasculitis with a chronic relapsing and remitting course.1 Ocular involvement, especially posterior segment involvement may cause significant visual loss. Therefore, immunomodulatory therapy is essential for the management of BU.2 The current therapeutic options for BU include systemic corticosteroids, azathioprine (AZA), cyclosporine (CSA), interferon alpha (IFNα), and anti-tumor necrosis factor-alpha (TNF-α) agents, including infliximab (IFX) and adalimumab (ADA).3 Failure or intolerance of these agents presents a challenge and the use of alternative biological agents may be needed such as other TNF-α antagonists, IL-1 antagonists anakinra, canakinumab, and gevokizumab, or the interleukin (IL)-6 inhibitor tocilizumab (TCZ).4–8
Clinical Follow-up of Patients with Behçet Uveitis after Discontinuation of Infliximab Therapy
Published in Ocular Immunology and Inflammation, 2022
Helin Ceren Köse, Nilüfer Yalçındağ
Behçet disease is a multigenetic inflammatory systemic disorder of unknown etiology characterized by recurrent episodes of oral aphthous ulcers, genital ulceration, skin lesions, and eye involvement. Patients with ocular involvement typically have recurrent episodes of uveoretinitis and chronic vascular inflammation presented by retinal and disc vascular leakage which can cause permanent damage to the retina and the optic nerve, leading to visual impairment.1–3 Therefore, the primary goal of Behçet uveitis management is controlling both recurrent ocular attacks and background vascular leakage for preservation of vision. Corticosteroids and immunosuppressive agents such as azathioprine and cyclosporine are generally used for the treatment of inflammation. However, these conventional therapies sometimes fail to control the acute episodes of intraocular inflammation in some patients.2,4
Demographic and Clinical Characteristics of Uveitis in Turkey: The First National Registry Report
Published in Ocular Immunology and Inflammation, 2018
F. Nilüfer Yalçındağ, Pınar C. Özdal, Yılmaz Özyazgan, Figen Batıoğlu, Ilknur Tugal-Tutkun
Investigators were instructed to record any established diagnosis based on clinical findings using the current international diagnostic criteria where applicable and/or laboratory investigations and consultations of other disciplines where available. The International Study Group for Behçet’s Disease criteria were used for the diagnosis of Behçet disease.44 The diagnosis of Vogt–Koyanagi–Harada (VKH) disease was based on the revised international criteria.45 However, categories of VKH diagnosis, as complete, incomplete, and probable, were not required to be registered. The registry system did not allow designation of “definite” or “presumed” categories for any of the diagnoses, including sarcoidosis, tuberculosis, and herpetic uveitis. Although tissue biopsy, culture, or PCR of ocular fluids might have been used for definitive diagnosis of these entities at some centers, the diagnoses were mostly presumed based on clinical grounds. Patients in whom diagnostic work-up had been performed, but a specific cause could not be identified or a specific diagnosis could not be made, were classified as having “presumed idiopathic autoimmune uveitis.” In patients in whom a former diagnosis was not present or could not be established at first presentation because of incomplete work-up, the diagnosis was recorded as “undefined uveitis.”
Related Knowledge Centers
- Aneurysm
- Contagious Disease
- Immunosuppressive Drug
- Inflammation
- Pathergy
- Visual Impairment
- Arthritis
- Corticosteroid
- Thrombus
- Genetic Disorder