Adult Primary Central Nervous System Vasculitis

An Update

Carlo Salvarani; Robert D. Brown; Gene G. Hunder


Curr Opin Rheumatol. 2012;24(1):46-52. 

In This Article

Abstract and Introduction


Purpose of review The aim of this review was to cover the recent major advances made in the fields of clinical assessment, diagnosis and treatment of adult primary central nervous system vasculitis (PCNSV).
Recent findings To prevent misdiagnosis, particularly with the reversible cerebral vasoconstriction syndromes, new criteria on the basis of the levels of certainty of the diagnosis of PCNSV have been proposed. Advances in the neuroimaging techniques visualizing the wall of intracranial blood vessels have improved the capacity to distinguish inflammatory from noninflammatory vascular lesions. These techniques could play in the future an important role in the diagnosis of PCNSV. Studies of larger numbers of cases have revealed a more varied histopathological inflammatory picture and disclosed an association with amyloid angiopathy. It has also been recognized that PCNSV is a heterogeneous disorder encompassing clinical subsets that differ in terms of prognosis and therapy. Finally, differently from earliest reports that suggested a poor prognosis with a fatal outcome in the majority of the cases, a large recent study from Mayo Clinic has described a more favorable course with good response to therapy and a favorable outcome in 81% of cases.
Summary Our better understanding of the PCNSV spectrum and its subsets will facilitate early recognition. This may facilitate earlier treatment and may prevent irreversible or even lethal outcomes.


Primary central nervous system vasculitis (PCNSV) is an uncommon and poorly understood form of vasculitis that it is limited to the brain and spinal cord.[1,2,3••] PCNSV represents the most frequent vasculitis involving the central nervous system (CNS).[4] The only available information on the incidence of PCNSV is from Olmsted County, Minnesota (USA) that was estimated at 2.4 cases per 1 000 000 person-years.[1] The neurological manifestations are diverse and nonspecific. Serological markers of inflammation are usually normal. Cerebrospinal fluid (CSF) is abnormal in approximately 80–90% of the cases. The diagnosis is unlikely in the presence of a normal brain MRI. Biopsy of CNS tissue showing vasculitis is the only definitive test; however, angiography is often used to confirm the diagnosis. Early recognition is important because treatment with glucocorticoids with or without cytotoxic drugs may prevent serious or even lethal outcomes. The differential diagnosis is broad and includes the reversible vasoconstriction syndromes, secondary cerebral vasculitis, malignancy and infections.

Modern recognition of PCNSV as a separate entity is generally dated to the mid-1950s when Cravioto and Feigin[5] described several cases with a 'noninfectious granulomatous angiitis' with a predilection for the nervous system. The term 'granulomatous angiitis of the nervous system' was applied because of the histopathologic findings observed in the arteries from initial cases.

Recently, major advances have been made in the field of PCNSV. Studies of larger numbers of cases have revealed a more varied histopathologic inflammatory picture and an association with amyloid angiopathy.[6–8] It has also become recognized that PCNSV is more heterogeneous than previously thought, encompassing clinical subsets that differ in terms of prognosis and therapy.[9–11,12••,13,14,15•] Finally, over the past few years, childhood PCNSV (cPCNSV) has been recognized as a possible cause of vascular strokes in children.[16] This review aimed to provide an update on the major advances made in adult PCNSV.


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