Management of primary and secondary central nervous system vasculitis

Salvarani, Carlo; Pipitone, Nicolò; Hunder, Gene G.

doi:10.1097/bor.0000000000000229

Cited by 29 publications

(8 citation statements)

References 63 publications

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“…Skin biopsies reveal polynuclear deposits in the walls of small and medium-sized blood vessels. Angiographic studies are also useful, as they show segmental dilatations and constrictions of blood vessels [ 36 ] The disease is usually treated with glucocorticoids, but a more aggressive course of treatment with glucocorticoids and cyclophosphamide is required in the presence of CNS or other vital organ involvement [ 41 ].…”

Section: Secondary Vasculitides Of the Central Nervous Systemmentioning

confidence: 99%

“…The occurrence of peripheral neuropathies is associated with a higher number of affected organs, increased ANCA titers and more severe disease [ 13 ]. CNS abnormalities occur in 7–11% of GPA patients [ 19 , 35 , 41 ]. The most common symptom is headache, followed by pachymeningitis and cranial nerve damage, with nerves II, VI and VII being the most commonly affected [ 35 ].…”

Section: Secondary Vasculitides Of the Central Nervous Systemmentioning

confidence: 99%

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Inflammatory Disorders of the Central Nervous System Vessels: Narrative Review

2022

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Inflammatory disorders of the central nervous system (CNS) vessels, also called CNS vasculitides, can cause substantial disability or even be fatal. Inflammation of the CNS vessels can be caused by primary angiitis of the CNS (PACNS), inflammatory cerebral amyloid angiopathy, or systemic inflammatory disorders. Clinical symptoms of these disorders are often non-specific, such as encephalopathy, cognitive and affective abnormalities, headache and focal neurological symptoms. Diagnostic workup includes a thorough neuropsychiatric examination, blood and cerebrospinal fluid analysis and magnetic resonance imaging (MRI) of the brain and its vessels. Biopsy of the brain remains the gold standard diagnostic test. Timely diagnosis and treatment initiation is of high importance, as it might prevent severe complications, such as ischemic and hemorrhagic stroke. In this review, we describe the specific characteristics of primary and secondary non-infectious CNS vasculitides which help to establish the diagnosis, discuss the peculiarities of the diagnostic workup and present current treatment recommendations.

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Section: Secondary Vasculitides Of the Central Nervous Systemmentioning

confidence: 99%

Section: Secondary Vasculitides Of the Central Nervous Systemmentioning

confidence: 99%

Inflammatory Disorders of the Central Nervous System Vessels: Narrative Review

2022

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“…Лабораторные показатели крови обычно не изменены, а в ликворе выявляются признаки воспаления (лимфоцитоз, наличие белка) и повышенное давление. При МРТ определяются множественные мелкие очаги, гиперинтенсивные в режимах Т2-ВИ и Т2-FLAIR, располагающиеся как в белом веществе, так и в коре головного мозга [29][30][31]. При контрастировании выявляются признаки линейного радиального накопления контраста очагами и вдоль сосудов, а также мягкими мозговыми оболочками, что отличает данный процесс от демиелинизации.…”

Section: клинический пример 4 синдром сусакаunclassified

Magnetic resonance imaging in the diagnosis of non-tumorous multifocal brain lesions mimicking multiple sclerosis

Krotenkova

Bryukhov

Konovalov

et al. 2021

Almanac of Clinical Medicine

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The diagnosis of multiple sclerosis (MS) is quite challenging due to its variable clinical manifestations and lack of a definitive test. Magnetic resonance imaging (MRI) is one of the tools to confirm the diagnosis and also helps in differential diagnosis with other disorders and in exclusion of MS-mimicking diseases. In this article, based on the analysis of clinical cases, we discuss the differential diagnosis of MS with the following non-tumorous multifocal brain lesions: vascular abnormalities caused by hypoxia and ischemia, cerebral autosomal dominant angiopathy with subcortical infarctions and leukoencephalopathy, Susac syndrome, primary angiitis of the central nervous system, and neurosarcoidosis. We present both MRI criteria for MS and disorders that have similar MRI signs, and additional clinical and laboratory data that is essential for correct diagnosis.

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“… 21 , 35 Biological agents have also been shown to be beneficial in difficult-to-treat patients with SACNS. 36 While in the cases of infection-associated vasculitis, antimicrobial or antiretroviral therapy should be first considered, 37 and adjunctive immunosuppressive therapy must be done with caution.…”

Section: Treatment Of Sacnsmentioning

confidence: 99%

A Closer Look at Angiitis of the central nervous system

Wan

2017

NSJ

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Angiitis of the central nervous system (ACNS) is a rather new disease entity that is defined as vascular inflammation limited to the central nervous system and was formally nominated in 1959. Etiologically, it can be divided into primary and secondary ACNS. However, the potential pathogenesis of ACNS remains unclear. The clinical presentation is variable, and there is no consensus concerning its diagnosis and management. Although the incidence is relatively low, ACNS is still a life-threatening condition. It is essential to get a comprehensive and updated understanding of the disease. In this paper, we reviewed the history, definition, classification, pathogenesis, and clinical manifestations of ACNS. In addition, we focused on the latest investigations and viewpoints regarding the diagnosis and treatment of ACNS.

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Management of primary and secondary central nervous system vasculitis

Abstract: Recognition of findings at diagnosis that predict the course or outcomes of PCNSV may serve as guide for therapy. Biological agents may provide benefit to difficult-to-treat patients with CNS involvement secondary to AAV and Behçet's disease.

Cited by 29 publications

References 63 publications

Inflammatory Disorders of the Central Nervous System Vessels: Narrative Review

Inflammatory Disorders of the Central Nervous System Vessels: Narrative Review

Magnetic resonance imaging in the diagnosis of non-tumorous multifocal brain lesions mimicking multiple sclerosis

A Closer Look at Angiitis of the central nervous system

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