ReviewCentral nervous system vasculitis in adults: An update
Introduction
Central nervous system (CNS) vasculitis is a rare disease that presents variable neurological manifestations depending on size and location of involved vessels within the nervous system. CNS vasculitis may be classified either by the size of the vessel involved (i.e. small, medium or large vessel vasculitis) or by the neuropathological findings (i.e. granulomatous, lymphocytic and necrotizing) [1], [2], [3]. CNS vasculitis may also be classified according to etiology as secondary, when associated with other systemic disorders such as autoimmune or infectious diseases, or as primary CNS vasculitis (PCNSV) when it is restricted to the CNS [1], [2], [4].
Suspicion of CNS vasculitis should be raised with the new onset of neurological symptoms associated with ischemic or hemorrhagic lesions in different stages in brain magnetic resonance imaging (MRI) affecting different vascular territories [5]. Since a wide variety of diseases may mimic CNS vasculitis and some of them do not benefit from immunosuppression, accurate diagnosis is of paramount importance.
Section snippets
Primary vasculitis of central nervous system
PCNSV is a rare single-organ vasculitis that affects small and medium vessels restricted to the CNS. All age groups may be affected, however a peak incidence in middle-aged men is observed [2], [6]. The diagnosis of PCNSV is challenging, since it requires extensive investigation to rule out various differential diagnoses [6].
There are no pathognomonic findings in PCNSV and the proposed diagnostic criteria are based on the demonstration of vasculitic findings either on angiography or on brain
Reversible cerebral vasoconstriction syndrome (RCVS)
RCVS is characterized by recurrent episodes of headache and brain vasoconstriction that resolve in months [30], [31]. It is most commonly seen in middle-aged women [6], patients usually present a monophasic disease, although 5% may experience recurrence [32]. Most patients present thunderclap headache (TCH), characterized by hyperacute severe headaches, reaching a maximum intensity in 1 min, with or without neurologic symptoms [33]; nonetheless approximately 15% develop other headache types or
Vasculitis secondary to connective tissue diseases
CNS involvement in connective tissue diseases (CTDs) occurs more frequently in systemic lupus erythematosus (SLE), Sjögren's syndrome (SS), mixed connective tissue disease (MCTD), and dermatomyositis [45].
CNS involvement in SLE occurs in more than 50% of patients [46]. Disruption of the blood-brain barrier is universal in neuropathology of SLE and the pathogenesis is multifactorial, involving various inflammatory cytokines, autoantibodies, and immune complexes, resulting in vasculopathic,
Conclusion
PCNSV is a challenging diagnosis due to the broad clinical manifestations and variable specificity and sensitivity of laboratory and imaging diagnostic tools. Brain biopsy should be performed whenever possible. Large vessel PCNSV should be managed with steroids and CYC whereas small vessel PCNSV with steroids alone due to recent data on prognosis. The most important differential diagnosis is RCVS, that may occur without thunderclap headache. Other differential diagnoses include secondary CNS
Conflict of interest
Drs. Dutra, Grinberg, Barsottini, and Silva de Souza report no disclosure. Dr. Appenzeller receives a Grant from Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq 304255/2015-7)
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