267. Differentiating primary central nervous system vasculitis from non-inflammatory intracranial vasculopathy
Creators: name: Moein   Amin,
name: Yuxuan   Jin
name: Shimoyam, Takashi
name: Uchino, Ken
name: Calabrese, Leonard
name: Hajj-Ali, Rula
Publisher: name: Zenodo
Year: 2022
Subjects: Vasculitis
ANCA
Abstracts
Giant Cell Arthritis
MPA
GPA
EGPA
Takayasu
IgA vasculitis

description: <strong>Background:</strong> The diagnosis of primary CNS vasculitis can be challenging and requires exclusion of other mimics. One of the major differential diagnoses for primary CNS vasculitis includes non-inflammatory vasculopathy and distinguishing the two can be challenging and potentially leading to invasive investigations. In this study, we aimed to investigate distinguishing primary central nervous system (CNS) vasculitis compared to non-inflammatory intracranial vasculopathy using features in clinical presentation and blood and cerebrospinal fluid (CSF) markers. <strong>Methods:</strong> We evaluated all cases enrolled in the prospective registry of CNS vasculopathy at a tertiary medical center. Final diagnoses were reviewed and patients with incomplete workup were excluded. Infectious vasculopathy, reversible cerebral vasoconstriction syndrome, autoimmune or inflammatory disease, and cardio-embolic disease were excluded since they may be more readily distinguished from primary CNS vasculitis. Several clinical features on first presentation such as gender, ethnicity, clinical presentation, past medical history, and medications in addition to serum and CSF findings were investigated for their ability to predict the final diagnosis. Logistic regression model was developed and validated using 3-fold cross validation. <strong>Results:</strong> Overall, 206 patients were identified who had completed the workup with a final diagnosis. Forty-eight patients with primary CNS vasculitis and 19 patients with non-inflammatory vasculopathies (large vessel atherosclerotic disease, small vessel disease, intracranial dissection, and Moya Moya disease) were included. A logistic regression model was evaluated with top 3 features including race, seizure on presentation, and CSF pleocytosis. The model predicted primary CNS vasculitis in validation cohort with sensitivity 72.2% and specificity 94.7%. Seizure upon presentation and CSF pleocytosis were associated with the diagnosis of CNS vasculitis and African-American race was associated with the diagnosis of non-inflammatory vasculopathy. <strong>Conclusions:</strong> Primary CNS vasculitis might be differentiated from non-inflammatory intracranial vasculopathy based on clinical presentation and CSF findings. Larger studies are warranted to validate these observations. <strong>Disclosures:</strong> none. Prelimimary data published here: https://acrabstracts.org/abstract/neurologic-involvement-in-anca-associated-vasculitis-data-from-multicenter-longitudinal-observational-study/ <strong>Table.</strong> Available demographic, clinical features, and laboratory features of patients with CNS vasculitis and noninflammatory vasculopathy including intracranial atherosclerotic disease, small vessel disease, dissection, Moyamoya disease, and unclear non-inflammatory vasculopathy. Statistically significant results are highlighted with *. N=number. CNS vasculitis Non-inflammatory P value <em>Gender</em> Male, N (%) N = 48 28 (58.3) N = 19 8 (42.1) 0.282 <em>Race</em> White, N (%) African American, N (%) Other, N (%) N = 48 43 (89.6) 1 (2.1) 4 (8.3) N = 19 13 (68.4) 4 (21.1) 2 (10.5) 0.063 0.021* 1.000 <em>Past medical history</em> Hyperlipidemia, N (%) Hypertension, N (%) Diabetes mellitus, N (%) Meningitis, N (%) N = 48 14 (29.2) 17 (35.4) 10 (20.8) 3 (6.3) N = 19 9 (47.4) 10 (52.6) 4 (21.1) 0 (0.0) 0.169 0.270 1.000 0.553 <em>Clinical presentation</em> Headache, N (%) Seizure, N (%) Visual symptoms, N (%) Aphasia or dysarthria, N (%) Focal sensorimotor deficit, N (%) Decreased level of consciousness, N (%) Memory impairment, N (%) Behavioral changes, N (%) N = 48 24 (50.0) 10 (20.8) 16 (33.3) 24 (50.0) 21 (43.8) 16 (33.3) 25 (52.1) 10 (20.8) N = 19 9 (47.4) 0 (0.0) 6 (31.6) 5 (26.3) 8 (42.1) 4 (21.1) 6 (31.6) 3 (15.8) 1.000 0.052 1.000 0.103 1.000 0.387 0.176 0.744 <em>Serum</em> Elevated C-reactive protein (&gt; 1.0 mg/dL), N (%) Antinuclear antibodies present, N (%) N = 47 6 (12.8) 3 (6.3) N = 19 1 (5.3) 2 (10.5) 0.663 0.621 <em>Vessel wall imaging MRI</em> Vessel wall enhancement on MRI, N (%) N = 24 10 (41.7) N = 15 10 (66.7) 0.191 CSF Leukocyte count, mononuclear leukocytes per µL median [IQR] Protein, mg/dL, median [IQR] Pleocytosis (≥ 5 mononuclear leukocytes per µL) (corrected for RBCs), N (%) N = 41 11 [2 – 28] 66 [43 – 89] 26 (63.4) N = 19 2 [0.5 – 3.5] 43 [31 – 53.5] 1 (5.3) &lt;0.001* 0.004* &lt;0.001* Elevated protein (&gt;45 mg/dL) (corrected for red blood cells), N (%) 28 (68.3) 7 (36.8) 0.027*