Inflammatory Disorders of the Central Nervous System Vessels: Narrative Review

Abstract

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.

Reversible Cerebral Vasoconstriction Syndrome and Raynaud’s Phenomenon: Is There a Link between the Pathogeneses of Their Underlying Complex Etiology? A Case Report and Literature Review

“…Due to the fact that our patient had RVCS and Raynaud’s phenomenon, the issue arises as whether or not there is a causal connection between the two conditions. Although the surgical history of breast implants in our case was not directly linked with RCVS in SLE, studies have also reported the use of immunosuppressant drugs, especially tocilizumab, prednisolone, and cyclophosphamide, and blood transfusion as a rare yet potential cause of RCVS when used as a treatment for connective tissue diseases [ 19 , 20 , 21 , 22 ]. Studies have also revealed the presence of RCVS in pediatric patients with SLE following immunosuppressive treatment for lupus nephritis, thereby mandating its early recognition, which may help adjust further treatment [ 23 , 24 ].…”

“…Both conditions may develop acute severe headaches, localize neurological impairments, and cause distinctive abnormalities in brain images [ 20 ]. Moreover, vasculitis is associated with persistent inflammation with insidious-onset headache, which progresses gradually [ 21 ]. In contrast, RCVS is associated with acute ‘thunderclap headaches,’ which are self-limiting and a typical characteristic, as demonstrated in our case [ 1 ].…”

“…Studies have also revealed the presence of RCVS in pediatric patients with SLE following immunosuppressive treatment for lupus nephritis, thereby mandating its early recognition, which may help adjust further treatment [ 23 , 24 ]. Calcineurin inhibitors have been identified as triggers for RCVS in the context of systemic lupus erythematosus (SLE), as they can cause damage to the endothelium of cerebral arteries and disrupt vascular tone control [ 19 , 20 , 21 , 22 ]. Moreover, existing data suggest that RCVS shares many common features with inflammatory CNS disorders that often require careful interpretation [ 22 ].…”

Induced Hypertension in the Management of Primary CNS Vasculitis

“…PCNSV is a life-threatening condition characterized by granulomatous inflammation within small cerebral blood vessels [1,2]. The disease is rare, with an estimated annual incidence of 2.4 cases per 1,000,000 person-years, and it affects people of all ages with slight predominance in the seventh decade of life [2,3].…”

“…PCNSV is a life-threatening condition characterized by granulomatous inflammation within small cerebral blood vessels [1,2]. The disease is rare, with an estimated annual incidence of 2.4 cases per 1,000,000 person-years, and it affects people of all ages with slight predominance in the seventh decade of life [2,3]. Diagnosis of PCNSV is challenging as it lacks standardized criteria, presents with subtle, nonspecific symptoms, and has a broad differential diagnosis, including intracranial atherosclerosis, reversible cerebral vasoconstriction syndrome, infectious and immune-mediated encephalitis, demyelinating syndromes, and neoplasms [1][2][3][4][5][6][7][8].…”