Diagnosis and follow-up evaluation of central nervous system vasculitis: an evaluation of vessel-wall MRI findings

Maximilian Patzig,Clemens Küpper,Florian Schöberl,Tobias Saam,Ozan Eren,Lars Kellert,Thomas Liebig,Robert Forbrig

doi:10.1007/s00415-021-10683-7

Maximilian Patzig, Clemens Küpper + Show 6 more

Open Access

https://doi.org/10.1007/s00415-021-10683-7

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Abstract

ObjectiveTo approach the clinical value of MRI with vessel wall imaging (VWI) in patients with central nervous system vasculitis (CNSV), we analyzed patterns of VWI findings both at the time of initial presentation and during follow-up.MethodsStenoocclusive lesions, vessel-wall contrast enhancement (VW-CE) and diffusion-restricted lesions were analyzed in patients with a diagnosis of CNSV. On available VWI follow-up, progression, regression or stability of VW-CE were evaluated and correlated with the clinical status.ResultsOf the 45 patients included, 28 exhibited stenoses visible on MR angiography (MRA-positive) while 17 had no stenosis (MRA-negative). VW-CE was found in 2/17 MRA-negative and all MRA-positive patients (p < 0.05). 79.1% (53/67) of stenoses showed VW-CE. VW-CE was concentric in 88.3% and eccentric in 11.7% of cases. Diffusion-restricted lesions were found more frequently in relation to stenoses with VW-CE than without VW-CE (p < 0.05). 48 VW-CE lesions in 23 patients were followed over a median time of 239.5 days. 13 VW-CE lesions (27.1%) resolved completely, 14 (29.2%) showed partial regression, 17 (35.4%) remained stable and 4 (8.3%) progressed. 22/23 patients received immunosuppressive therapy for the duration of follow-up. Patients with stable or progressive VW-CE were more likely (p < 0.05) to have a relapse (14/30 cases) than patients with partial or complete regression of VW-CE (5/25 cases).ConclusionConcentric VW-CE is a common finding in medium/large-sized vessel CNSV. VW-CE might represent active inflammation in certain situations. However, follow-up VWI findings proved ambiguous as persisting VW-CE despite immunosuppressive therapy and clinical remission was a frequent finding.

Highlights

Central nervous system (CNS) vasculitis is a rare disease characterized by different etiologies, heterogeneous findings and a lack of definite diagnostic markers
The role of vessel wall imaging both regarding the diagnosis of CNS vasculitis and monitoring disease activity, in response to immunosuppression, remains largely unproven. It is for these reasons that we retrospectively evaluated clinical and radiological data of patients with CNS vasculitis treated at our institution, aiming to contribute data on the pattern of vessel wall imaging findings both at the time of initial presentation and at follow-up
In 28 of the patients with large/medium vessel CNS vasculitis, stenoses or irregularities of intracranial arteries were visualized on MR angiography (“magnetic resonance angiography (MRA)-positive”) while two patients showed abnormalities of medium-sized arteries on digital subtraction angiography (DSA) only (“MRA-negative”)

Summary

Introduction

Central nervous system (CNS) vasculitis is a rare disease characterized by different etiologies, heterogeneous findings and a lack of definite diagnostic markers. It poses great challenges regarding both diagnosis and treatment [1,2,3,4,5,6]. Along with clinical and laboratory findings, imaging is crucial in the work-up of CNS vasculitis [7] Findings of both digital subtraction angiography (DSA) and conventional magnetic resonance imaging (MRI) including magnetic resonance angiography (MRA) are unspecific regarding CNS vasculitis [8,9,10]. Even biopsy has a limited sensitivity with a relevant rate of false negative results, when only medium- and/ or large-sized vessels are affected [13,14,15,16,17]

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