Elsevier

Journal of Autoimmunity

Volumes 48–49, February–March 2014, Pages 149-152
Journal of Autoimmunity

Diagnosis and classification of central nervous system vasculitis

https://doi.org/10.1016/j.jaut.2014.01.007Get rights and content

Abstract

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.

Introduction

Central nervous system (CNS) vasculitis refers to a broad array of diseases that result in inflammation and destruction of the blood vessels of brain, spinal cord and the meninges. CNS vasculitis is classified into primary and secondary. The vasculitis is primary when it is confined to the central nervous system with no involvement of other systems; thus it is referred to as primary angiitis of the CNS (PACNS). CNS vasculitis is considered secondary when it occurs in the context of a systemic inflammatory or infectious process. Examples of diseases that may secondarily affect the CNS in a vasculitic pattern include connective tissue disease, systemic vasculitis, chronic inflammatory disease, or infectious disease such as Varicella zoster and others.

Section snippets

Historical background

PACNS was initially reported in 1959, when Cravioto and Feigin [1] reported pathologic findings of CNS vasculitis when examining brain autopsies. The disease was considered uniformly fatal until reports of successful treatment emerged in the 1980s [2]. Since then, the medical literature has documented a rapid growth in the number of cases along with significant advances in diagnostic modalities and a much greater awareness of the disease [3].

Epidemiology

PACNS remains a rare disorder. A retrospective analysis of 101 cases revealed that the average annual incidence rate of PACNS is 2.4 cases per one million person-years [4]. The disease has been reported more commonly in white males with median age at onset being 50 years [4], [5], [6]. Children have also been affected [7], [8], [9]. The epidemiology of secondary CNS vasculitis depends on the underlying primary disease; ischemic strokes and cerebral hemorrhage occur in 10% of patients with

Diagnostic criteria and clinical features

Diagnostic criteria for PACNS were proposed over 20 years ago by Calabrese et al. [5]; and although un-validated, have been used widely for clinical reporting and investigation (Table 1). The diagnosis of secondary CNS vasculitis is usually based on a supporting pattern of vasculitic activity directly by tissue biopsy or indirectly by radiological modalities in the setting of a systemic inflammatory or infectious disease.

Calabrese's criteria include the following: (1) the presence of an

Diagnosis of PACNS

An accurate and timely diagnosis of PACNS remains one of the most challenging diagnostic tasks in rheumatology. The absence of specific serological tests and the limited sensitivity and specificity of cerebrospinal fluid (CSF) examination, cerebral angiography, and brain biopsy limit early and rapid diagnosis. Epidemiologically, PACNS is far less common than its mimics further underlying the critical need to rule out mimics. Multiple common scenarios exist where PACNS is frequently considered;

Differential diagnosis

Differential diagnosis of PACNS is protean (Table 2). Multifocal cerebral thromboembolisms resulting from endocarditis, left atrial myxoma, carotid atherosclerosis, antiphospholipid antibody syndrome and other hypercoagulable disorders should be excluded very early on during the evaluation.

Radiologically, diseases that have the potential of imitating the MRI findings of PACNS include brain tumors (e.g. intravascular lymphoma, gliomatosis cerebri), demyelinating disease, and genetic conditions

Summary

The diagnostic task for CNS vasculitis is challenging and should include a team of expert diagnosticians in many fields. The advancement in diagnostic modalities demystified many of the mimics of CNS vasculitis. Studies on the diagnostic and follow up roles of HR-MRI are such an example. However, a great need exists to expatiate on different aspects of CNS vasculitis such as epidemiologic properties, pathogenesis, diagnostic tools, and management strategies. Substantial work is currently

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