The Experts below are selected from a list of 109953 Experts worldwide ranked by ideXlab platform
Leonard H Calabrese - One of the best experts on this subject based on the ideXlab platform.
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diagnosis and classification of central nervous system Vasculitis
Journal of Autoimmunity, 2014Co-Authors: Rula A Hajjali, Leonard H CalabreseAbstract:Central nervous system Vasculitis is one of the foremost diagnostic challenges in rheumatology. It results in inflammation and destruction of the vasculature within the CNS. When Vasculitis is confined to brain, meninges or spinal cord, it is referred to as primary angiitis of the CNS. Secondary CNS Vasculitis occurs in the setting of a systemic Vasculitis, auto-inflammatory or infectious disease. Prompt and accurate diagnosis of CNS Vasculitis is essential to prevent irreversible brain damage, and to secure precise treatment decisions. Progressive debilitating and unexplained neurological deficits, associated with abnormal cerebrospinal fluid is the typical picture of the disease. Biopsy of the brain remains the gold standard diagnostic test. The differential diagnosis of CNS Vasculitis is highly diverse with a broad array of mimics at the clinical, radiographic and angiographic levels.
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central nervous system Vasculitis
Current Opinion in Rheumatology, 2009Co-Authors: Rula A Hajjali, Leonard H CalabreseAbstract:Central nervous system (CNS) Vasculitis is an uncommon disease that results in Vasculitis of the CNS or spinal cord and a rare cause of stroke. CNS Vasculitis is categorized into primary angiitis of the CNS (PACNS) or secondary CNS Vasculitis and represents a wide spectrum of neurological signs such as headache, seizure, mental changes, and focal neurological deficit. Although no diagnostic criteria has been validated prospectively, the diagnosis of CNA Vasculitis is based on a combination of laboratory findings such as elevations in cerebrospinal fluidprotein and white blood cells, imaging such as magnetic resonance imaging and cerebral angiography, and histology from a brain biopsy. A variety of mimics of CNS Vasculitis need to be ruled out prior to the diagnosis being made, including reversible cerebral vasoconstriction syndrome, infectious causes and malignancy. Prompt diagnosis of CNS Vasculitis and urgent treatment with glucoroticoids or immunosuppressive agents such as cyclophosphamide are critical to prevent poor outcome.
Rula A Hajjali - One of the best experts on this subject based on the ideXlab platform.
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diagnosis and classification of central nervous system Vasculitis
Journal of Autoimmunity, 2014Co-Authors: Rula A Hajjali, Leonard H CalabreseAbstract:Central nervous system Vasculitis is one of the foremost diagnostic challenges in rheumatology. It results in inflammation and destruction of the vasculature within the CNS. When Vasculitis is confined to brain, meninges or spinal cord, it is referred to as primary angiitis of the CNS. Secondary CNS Vasculitis occurs in the setting of a systemic Vasculitis, auto-inflammatory or infectious disease. Prompt and accurate diagnosis of CNS Vasculitis is essential to prevent irreversible brain damage, and to secure precise treatment decisions. Progressive debilitating and unexplained neurological deficits, associated with abnormal cerebrospinal fluid is the typical picture of the disease. Biopsy of the brain remains the gold standard diagnostic test. The differential diagnosis of CNS Vasculitis is highly diverse with a broad array of mimics at the clinical, radiographic and angiographic levels.
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central nervous system Vasculitis
Current Opinion in Rheumatology, 2009Co-Authors: Rula A Hajjali, Leonard H CalabreseAbstract:Central nervous system (CNS) Vasculitis is an uncommon disease that results in Vasculitis of the CNS or spinal cord and a rare cause of stroke. CNS Vasculitis is categorized into primary angiitis of the CNS (PACNS) or secondary CNS Vasculitis and represents a wide spectrum of neurological signs such as headache, seizure, mental changes, and focal neurological deficit. Although no diagnostic criteria has been validated prospectively, the diagnosis of CNA Vasculitis is based on a combination of laboratory findings such as elevations in cerebrospinal fluidprotein and white blood cells, imaging such as magnetic resonance imaging and cerebral angiography, and histology from a brain biopsy. A variety of mimics of CNS Vasculitis need to be ruled out prior to the diagnosis being made, including reversible cerebral vasoconstriction syndrome, infectious causes and malignancy. Prompt diagnosis of CNS Vasculitis and urgent treatment with glucoroticoids or immunosuppressive agents such as cyclophosphamide are critical to prevent poor outcome.
Jacek Musiał - One of the best experts on this subject based on the ideXlab platform.
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Cutting Edge Issues in the Churg–Strauss Syndrome
Clinical Reviews in Allergy & Immunology, 2013Co-Authors: Wojciech Szczeklik, Bogdan Jakieła, Dariusz Adamek, Jacek MusiałAbstract:Churg–Strauss syndrome (CSS) is a rare systemic small-vessel Vasculitis that develops in the background of bronchial asthma, which is characterized by eosinophilia and eosinophilic infiltration of various tissues. It belongs to the group of antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides. The triggering factors and pathogenesis of CSS are still unknown. The possible role of eotaxin-3 and CCR4-related chemokines in selective recruitment of eosinophils to the target tissues in CSS has been recently suggested, but the role of eosinophilic inflammation in the development of vasculitic lesions is not completely understood. From the clinical view, two distinct phenotypes of the disease are slowly emerging depending on the ANCA-positivity status. Glucocorticoids are still the mainstay of treatment; however, data are accumulating regarding the beneficial role of novel immunosuppressants and biologic compounds, especially in patients with poorer prognosis.
Adi Vaknin-dembinsky - One of the best experts on this subject based on the ideXlab platform.
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Central Nervous System Vasculitis
Current Treatment Options in Neurology, 2013Co-Authors: Marc Gotkine, Adi Vaknin-dembinskyAbstract:Opinion statementA patient presenting with suspected primary central nervous system (CNS) Vasculitis is often difficult to manage. Once the diagnosis is suspected, the clinical course may already be severe and warrant timely aggressive treatment with potentially toxic drugs. Definitive diagnosis is often delayed, requiring specialized tests that are only possible in certain centers and sometimes prove to be inconclusive. The need to make a confident diagnosis before starting powerful and potentially toxic immunosuppression, often conflicts with the desire to initiate timely treatment aimed at preventing irreversible neurological dysfunction. The heterogeneity and rarity of this condition is a major obstacle to conducting properly designed, double blinded, placebo-controlled studies. There is no Class I evidence for any form of treatment in these situations, and management guidelines represent expert opinion, based primarily on trials investigating therapies affecting systemic conditions with a CNS vasculitic component. Cyclophosphamide is still the main immunosuppressive drug used during active disease in combination with high dose steroids. Rituximab may be an equally efficacious, less toxic alternative, but its use is still limited due to its cost.
Susanne M Benseler - One of the best experts on this subject based on the ideXlab platform.
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central nervous system Vasculitis in children
Current Opinion in Rheumatology, 2008Co-Authors: Jorina Elbers, Susanne M BenselerAbstract:Central nervous system (CNS) Vasculitis is an increasingly recognized, often devastating inflammatory brain disease of children and adults. In primary or isolated CNS Vasculitis/angiitis of childhood (cPACNS), the vascular inflammation is limited to the brain and spinal cord. Secondary CNS Vasculitis occurs in a variety of conditions including infections, collagen vascular diseases, systemic vasculidities, and malignancies. Mimics of CNS Vasculitis in children include dissections and noninflammatory vasculopathies. Diagnosis of primary CNS Vasculitis in both adults and children is based on the Calabrese criteria. This review summarizes recent data on CNS Vasculitis in children; reviews the clinical spectrum at presentation and the role of laboratory tests, neuroimaging, and brain biopsy; and discusses treatment strategies, outcome data, and overlapping conditions of cPACNS.
