Introduction: Primary central nervous system vasculitis (PCNSV) is a rare and poorly understood disease of the brain and spinal cord. Cerebral angiography is the radiological gold standard for diagnosis of PCNSV in patients with compatible clinical findings. However, advances in the quality of noninvasive neuroimaging techniques of cerebral and spinal vasculature such as magnetic resonance angiography (MRA) and computed tomography angiography (CTA) may obviate the need for invasive catheter angiography. Reviewing our recent institutional experience, we assess the utility of performing a digital subtraction angiogram (DSA) in the management of suspected PCNSV.
Methods: Data from 33 consecutive patients who underwent DSA in addition to either MRA or CTA for suspected PCNSV at Jackson Memorial Hospital, Miami FL between 2011 and 2016 were retrospectively collected. Patient demographics, presenting symptoms, and noninvasive imaging results were recorded. Diagnosis and medical management prior and subsequent to DSA were compared.
Results: In 70% (n=23) of patients, both noninvasive imaging (MRA/CTA) and DSA failed to show evidence of PCNSV. Despite negative imaging studies, 4 of these patients (17%) were treated empirically for PCNSV with immunosuppressants based on clinical symptoms and laboratory findings. Additionally, 12% of patients (n=4) demonstrated non-diagnostic mild irregularities on noninvasive imaging. Subsequent DSA confirmed a diagnosis of PCNSV in 3 of these patients; however, all were started on immunosuppression thereafter. CTA/MRA demonstrated clear evidence of PCNSV in 18% of cases (n=6). Subsequent DSA revealed normal cerebral angiograms on 2 such patients and confirmed PCNSV in 4 patients. Of these 6 patients, only one patient did not receive immunosuppressive treatment after the finding of a normal DSA.
Conclusions: Invasive catheter-based angiography may be of limited benefit in the diagnosis and management of PCNSV when considered in the context of clinical and laboratory findings and MRA or CTA results.
Patient Care: This research will contribute to creating a diagnostic algorithm for PCNSV which reliably directs treatment and minimizes the risks of invasive procedures.
Learning Objectives: By the conclusion of this session, participants should be able to: 1) Identify the diagnostic work-up for PCNSV 2) Discuss in small groups the pros and cons of each diagnostic tool 3) Identify an effective strategy for when to use invasive angiography in the diagnosis and management of PCNSV