Table 2.
Characteristics of the study population
| n = 45, f/m 1.5:1, 58 y [19–75] (median [range]) | ||
|---|---|---|
| Medium/large-vessel vasculitis | 66.7% (n = 30) f/m 1.3:1 |
55 y [19–75] |
| Etiology/diagnosis | ||
| PACNS | 63.3% (n = 19) | 52 y [19–75] |
| Systemic autoimmune vasculitis with CNS involvement | 26.7% (n = 8) | 60.5 y [38–74] |
| Giant cell arteriitis | 16.7% (n = 5) | 66 y [59–74] |
| Systemic lupus | 3.3% (n = 1) | 38 y |
| Unclassified systemic vasculitis | 6.6% (n = 2) | 41, 52 y |
| (Para)infectious CNS vasculitis | 10.0% (n = 3) | 57 y [53–68] |
| HIV/Lues | 3.3% (n = 1) | 68 y |
| HSV-1 | 3.3% (n = 1) | 57 y |
| HHV-6 | 3.3% (n = 1) | 53 y |
| Type of onset | ||
| Acute (stroke-like) | 66.7% (n = 20) | |
| Subacute (days to weeks) | 33.3% (n = 10) | |
| Chronic progressive (months) | 0.0% (n = 0) | |
| Symptoms at onset | ||
| Headache | 43.3% (n = 13) | |
| Neuropsychiatric complaints | 16.7% (n = 5) | |
| Epilepsy/seizures | 0.0% (n = 0) | |
| (Multi)focal deficits |
66.7% (n = 20) NIHSS 2 [0–12] |
|
| Relapses (of n = 21 patients with 48 follow-up evaluations) | ||
| Overall number of relapses | 33.3% (16/48) | |
| Clinical worsening and new DWI lesions | 14.5% (7/48) | |
| New DWI lesions only | 6.3%(3/48) | |
| Clinical worsening only | 12.5% (6/48) | |
| Number of patients with relapses | 52.4% (11/21) | |
| Clinical worsening and new DWI lesions | 33.3% (7/21) | |
| New DWI lesions only | 4.8% (1/21) | |
| Clinical Worsening only | 19.0% (4/21) | |
| Immunosuppression (of n = 21 patients with 48 follow-up evaluations) | ||
| Overall | 95.0% (20/21) | |
| Steroids | 95.0% (20/21) | |
| Other immunotherapies |
66.7% (14/21) Cyc (n = 8), Aza (n = 5), MTX (n = 3), RTX (n = 2), Toc (n = 2) |
|
| Combination of steroids plus one additional immunosuppressant | 71.4% (15/21) | |
| Combination of steroids plus > 1 additional immunosuppressant | 9.5% (2/21) | |
| Increase of immunotherapies in relapse | 100% (16/16 relapses in 11/11 patients) | |
| Small vessel vasculitis | 33.3% (n = 15) f/m 2:1 |
62 y [24–75] |
|---|---|---|
| Etiology/diagnosis | ||
| PACNS | 46.7% (n = 7) | 62 y [46–74] |
| ANCA-associated systemic vasculitis | 20.0% (n = 3) | 73 y [44–75] |
| mPA | 13.3% (n = 2) | 73, 75 y |
| GPA | 6.7% (n = 1) | 44 y |
| Systemic lupus | 13.3% (n = 2) | 24, 69 y |
| Other autoimmune etiology | 20.0% (n = 3) | 62 y [54–67] |
| CREST | 6.7% (n = 1) | 67 y |
| Sjögren | 6.7% (n = 1) | 54 y |
| CAPS | 6.7% (n = 1) | 61 y |
| Type of onset | ||
| Acute (stroke-like) | 26.7% (n = 4) | |
| Subacute (days to weeks) | 66.7% (n = 10) | |
| Chronic progressive (months) | 6.7% (n = 1) | |
| Symptoms | ||
| Headache | 53.3% (n = 8) | |
| Neuropsychiatric complaints | 46.7% (n = 7) | |
| Epilepsy/seizures | 6.7% (n = 1) | |
| (Multi)focal deficits |
80.0% (n = 12) NIHSS 3 [0–7] |
|
| Relapses (of n = 2 patients with 7 follow-up evaluations) | ||
| Overall number of relapses | 42.9% (3/7) | |
| Clinical worsening and new DWI lesions | 14.2% (1/7) | |
| New DWI lesions only | 0% (0/7) | |
| Clinical worsening only | 28.6% (2/7) | |
| Number of patients with relapses | 100% (2/2) | |
| Clinical worsening and new DWI lesions | ||
| New DWI lesions only | 0% (0/2) | |
| Clinical worsening only | 50.0% (1/2) | |
| Immunosuppression | ||
| Overall | 100% (2/2) | |
| Steroids | 100% (2/2) | |
| Other immunotherapies |
100% (2/2) RTX (n = 1), MTX (n = 1), Anakinra (n = 1) |
|
| Combination of steroids plus one additional immunosuppressant | 50% (1/2) | |
| Combination of steroids plus > 1 additional immunosuppressant | 50% (1/2) | |
| Increase of immunotherapies in relapse | 100% (3/3 relapses in 2/2 patients) | |
f female, m male, y years, Aza azathioprine, CAPS cryoporine-associated periodic (fever) syndrome, CREST calcinosis cutis/raynaud syndrome/esophagus involvement/sclerodermia/teleangiectasia, DWI diffusion-weighted imaging, GPA granulomatosis with polyangiitis, HHV-6 human herpes virus type 6, HIV human immunodeficiency virus, HSV herpes simplex virus, mPA microscopic polyangiitis, MTX methotrexate, MMF mycophenolate-mofetil, PACNS primary angiitis of the central nervous system, RTX rituximab, Toc tocilizumab