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Laryngo- Rhino- Otologie Jun 2020Behçet's syndrome (BS) is classified as a variable vessel vasculitis. The clinical picture is very diverse and usually requires interdisciplinary collaboration....
Behçet's syndrome (BS) is classified as a variable vessel vasculitis. The clinical picture is very diverse and usually requires interdisciplinary collaboration. Pathogenetically, BS seems to take a middle position between a polygenic autoinflammatory disease and an autoimmune disease. New EULAR recommendations were issued in 2018. The therapy depends on which organs are most affected. Since 2016, adalimumab has been approved for the treatment of posterior ocular involvement. Infliximab, interferon a2a, interleukin-1 antagonists and apremilast may be alternative therapies.
Topics: Behcet Syndrome; Humans
PubMed: 32557459
DOI: 10.1055/a-1076-9742 -
Clinical and Experimental Medicine Dec 2023Behçet's syndrome is a recurring inflammatory multiorgan disorder affecting the skin, mucosa, eyes, joints, stomach, and central nervous system. Behçet's syndrome... (Review)
Review
Behçet's syndrome is a recurring inflammatory multiorgan disorder affecting the skin, mucosa, eyes, joints, stomach, and central nervous system. Behçet's syndrome epidemiology varies greatly among populations (0.64-420/100,000), and Behçet's syndrome has gained increasing international acclaim in the recent 50 years due to raising awareness of the syndrome, although it is rare in most population. In addition to the unclear etiology of the syndrome, the diagnosis of Behçet's syndrome is complicated by a vague clinical presentation, phenotypic heterogeneity and/or incomplete representation, and the lack of any specific laboratory, radiographic, or histological findings. There exists a dire need to elucidate factors that contribute to disease pathogenesis and/or are associated with clinical features of Behçet's syndrome and the classification of different forms of the syndrome. The identification of such molecular, cellular, and/or clinical factors are crucial for timely diagnosis and efficacious management of Behçet's syndrome. We discuss recent advances in the clinical diagnosis of Behçet's syndrome and related contributions of genetics, epigenetics, microbiome, inflammasomes, and autoantibodies to the improved diagnosis, management, and understanding of Behçet's syndrome.
Topics: Humans; Behcet Syndrome; Skin
PubMed: 37897656
DOI: 10.1007/s10238-023-01226-7 -
Revista de La Facultad de Ciencias... Mar 2022To provide a complete picture and to improve understanding of the serpiginous-like choroiditis caused by Mycobacterium Tuberculosis. (Review)
Review
OBJECTIVE
To provide a complete picture and to improve understanding of the serpiginous-like choroiditis caused by Mycobacterium Tuberculosis.
MATERIALS AND METHODS
Literature review of serpiginous-like choroiditis. A number of scientific search engines were searched including Medline (PubMed), Scielo, and Cochrane Library. Using MeSH and DeCS terms.
RESULTS
107 articles were obtained, of which 44 met inclusion criteria.
DISCUSSION
serpiginous-like choroiditis is described as its etiology, pathogenesis, ocular findings, diagnosis, differential diagnosis and treatment.
CONCLUSIONS
Modern studies are needed to understand the pathophysiology, new diagnostic strategies, and future treatments of serpiginous-like choroiditis
Topics: Choroiditis; Diagnosis, Differential; Humans
PubMed: 35312256
DOI: 10.31053/1853.0605.v79.n1.32223 -
Brain and Nerve = Shinkei Kenkyu No... May 2021Neuro-Behçet's disease accounts for 10-20% cases of Behçet's disease, and patients manifest neurological symptoms due to parenchymal inflammation in the central... (Review)
Review
Neuro-Behçet's disease accounts for 10-20% cases of Behçet's disease, and patients manifest neurological symptoms due to parenchymal inflammation in the central nervous system. Neuro-Behçet's disease is classified into two types (acute and chronic progressive) based on clinical symptoms and therapeutic responsiveness. This review aimed to summarize the acute type of neuro-Behçet's disease according to the guidelines of the Japanese Behçet's Disease Research Committee.
Topics: Acute Disease; Behcet Syndrome; Central Nervous System; Humans; Magnetic Resonance Imaging
PubMed: 34006689
DOI: 10.11477/mf.1416201799 -
Ocular Immunology and Inflammation May 2023Sympathetic ophthalmia (SO) is rare, bilateral granulomatous panuveitis that typically occurs following penetrating or perforating ocular trauma or surgery. This review... (Review)
Review
INTRODUCTION
Sympathetic ophthalmia (SO) is rare, bilateral granulomatous panuveitis that typically occurs following penetrating or perforating ocular trauma or surgery. This review aims to provide an update on the etiopathogenesis, clinical presentations, diagnosis and treatment of SO.
METHODS
Reports cited in MEDLINE database, that analyzed SO in at least 5 patients, published prior to December 1st, 2021 were included.
RESULTS
Initially, SO was associated with penetrating ocular trauma, however, various studies reported an increased incidence of SO after surgical procedures including vitreoretinal surgeries. Multimodal imaging including fluorescein and indocyanine green angiography, optical coherence tomography (OCT) and OCT angiography have added further insights into the understanding of SO. While pulse dose corticosteroids & immunosuppressive drugs are still the treatment of choice, TNF-α blockers & other biologic drugs represent new promising agents.
CONCLUSION
There is a growing pool of evidence in understanding the pathogenesis of SO. Novel treatment options have provided better prognosis for this potentially blinding condition.
Topics: Humans; Ophthalmia, Sympathetic; Immunosuppressive Agents; Immunologic Factors; Prognosis; Tomography, Optical Coherence; Eye Injuries; Fluorescein Angiography
PubMed: 35579612
DOI: 10.1080/09273948.2022.2058554 -
Revue Des Maladies Respiratoires Jun 2022Behcet's disease (BD) is a multisystemic vasculitis involving arteries and veins of all sizes. While joint and dermatological manifestations are the most common features... (Review)
Review
Behcet's disease (BD) is a multisystemic vasculitis involving arteries and veins of all sizes. While joint and dermatological manifestations are the most common features of BD and are associated with a good prognosis; vascular involvement, remains the principal cause of death. Arterial manifestations occur in 5-10% of cases and manifest as occlusion/thrombosis or aneurysms. Arterial aneurysms are likely multiple and the most common sites are pulmonary arteries, aorta and arteries of lower limbs. Parenchymal involvement is less frequent and may manifest as consolidation or nodules, which may evolve to excavation. Aneurysms may occur at the sites of arterial puncture; then, non-traumatic techniques are favored. Patients with arterial manifestations may present with fever and increased inflammatory markers. Artery damage is rare, serious, and may result in massive hemoptysis. The prognosis of pulmonary artery aneurysms is severe (mortality estimated up to 26%) but has been improved by earlier diagnosis and the introduction of immunosuppressants. Treatment of severe arterial manifestations is based on high-dose corticosteroids along with cyclophosphamide or anti-TNF antagonists. Anticoagulation could be added to immunosuppressants in case of venous thrombosis if a coexisting pulmonary aneurysm is ruled out. Endovascular treatment should be performed in case of severe symptomatic pulmonary aneurysms, along with an adequate medical management. Long-term maintenance therapy of these severe forms is of paramount importance because of relapse risk (40% at five years).
Topics: Aneurysm; Behcet Syndrome; Humans; Immunosuppressive Agents; Pulmonary Artery; Tumor Necrosis Factor Inhibitors
PubMed: 35659162
DOI: 10.1016/j.rmr.2022.04.010 -
Frontiers in Public Health 2023Cytomegalovirus anterior uveitis is the most common ocular inflammatory disease caused by cytomegalovirus infection. It mainly occurs in middle-aged males with competent... (Review)
Review
Cytomegalovirus anterior uveitis is the most common ocular inflammatory disease caused by cytomegalovirus infection. It mainly occurs in middle-aged males with competent immunologic function, and the incidence is higher in Asia. The clinical manifestations vary from Posner-Schlossman syndrome and corneal endotheliitis to Fuchs uveitis syndrome, and are often accompanied by intraocular hypertension. Secondary glaucoma is a potentially blinding ocular complication with a pathogenesis that includes complicated immunological factors, intraocular inflammation, different types of angle abnormalities, and the administration of steroids, which may result in physical discomfort and visual impairment. Diagnostic tests, such as the polymerase chain reaction, optical coherence tomography, ocular microscopy, and confocal microscopy, might help in identifying anterior uveitis caused by other viruses. Combinations of antiviral medications and anti-inflammatory agents are effective treatments. If pharmacological therapy cannot reduce intraocular pressure or slow the progression of glaucomatous optic neuropathy, surgical intervention is required as a last resort.
Topics: Male; Middle Aged; Humans; Cytomegalovirus; Glaucoma; Uveitis, Anterior; Eye; Cytomegalovirus Infections
PubMed: 36935679
DOI: 10.3389/fpubh.2023.1117412 -
Current Rheumatology Reports Jan 2024We aimed to highlight disease-related and treatment-related complications of Behçet syndrome (BS) based on previous and recent studies and our own experience. (Review)
Review
PURPOSE OF REVIEW
We aimed to highlight disease-related and treatment-related complications of Behçet syndrome (BS) based on previous and recent studies and our own experience.
RECENT FINDINGS
The Behçet's Disease Overall Damage Index is a newly developed instrument to assess damage in BS. Validation studies showed that damage is already present in some patients at diagnosis and continues to progress during the follow-up, mainly related to treatment complications. Nervous system and eye involvement are important causes of long-term disability. Cyclophosphamide seems to be associated with infertility and an increased risk of malignancies among BS patients, prompting the consideration of shortening the treatment duration. Flares in mucocutaneous manifestations have been reported with tocilizumab, and de novo BS manifestations with secukinumab therapy. Earlier diagnosis and treatment are essential to prevent disease-related damage in BS. Treatment-related complications seem to be the leading cause of damage during the disease course.
Topics: Humans; Behcet Syndrome; Cyclophosphamide; Risk Factors; Disease Progression
PubMed: 37995045
DOI: 10.1007/s11926-023-01124-7 -
Seminars in Arthritis and Rheumatism Dec 2019Acute anterior uveitis is the most common extra-articular clinical manifestation of spondyloarthropathy. Rheumatologists should be aware of uveitis, know how it... (Review)
Review
Acute anterior uveitis is the most common extra-articular clinical manifestation of spondyloarthropathy. Rheumatologists should be aware of uveitis, know how it presents, understand the differential diagnosis of uveitis and arthritis, and be familiar with the role of systemic medications in the treatment or prevention of uveitis.
Topics: Acute Disease; Animals; Diagnosis, Differential; Glucocorticoids; Humans; Spondylarthritis; Uveitis, Anterior
PubMed: 31779847
DOI: 10.1016/j.semarthrit.2019.09.014 -
Turkish Journal of Ophthalmology Jun 2020Behçet's disease is a chronic, multisystem inflammatory disorder characterized by relapsing inflammation. Although its etiopathogenesis has not yet been clarified, both... (Review)
Review
Behçet's disease is a chronic, multisystem inflammatory disorder characterized by relapsing inflammation. Although its etiopathogenesis has not yet been clarified, both the adaptive and innate immune systems, genetic predisposition, and environmental factors have all been implicated. It is more frequent and more severe in males in the third and fourth decades of life. The eye is the most frequently involved organ in the course of the disease. Ocular involvement (Behçet's uveitis) is characterized by bilateral recurrent non-granulomatous panuveitis and occlusive retinal vasculitis. Recurrent inflammatory episodes in the posterior segment may lead to permanent vision loss due to irreversible retinal damage and complications such as macular scarring, macular atrophy, and optic atrophy. Early and aggressive immunomodulatory treatment and the use of biologic agents when needed are crucial for preventing recurrences and improving visual prognosis.
Topics: Behcet Syndrome; Biological Factors; Disease Management; Glucocorticoids; Humans; Immunosuppressive Agents; Prognosis; Recurrence; Retina
PubMed: 32631005
DOI: 10.4274/tjo.galenos.2019.60308