The objectives of this study are to compare the initial clinical, laboratory, and imaging features in
primary central nervous system vasculitis (PCNSV) vs secondary
central nervous system vasculitis (SCNSV) and follow up
after treatment with intravenous
cyclophosphamide (IV-CYC) plus glucocorticosteroids (GCS):
methylprednisolone (MP). Neurological, laboratory, and neuroimaging findings were analyzed in PCNSV and SCNSV patients. Cerebral biopsy (CB) was performed in nine patients. Both groups received at onset MP plus IV-CYC for 6 months, followed by bimonthly IV-CYC plus
prednisone (PND) for 12 months. All patients were followed during 36 months. Thirty patients were included (12 PCNSV and 18 SCNSV). Focal and non-focal
neurological manifestations were similar in both groups,
headache being the most frequent manifestation in both groups.
Fatigue, myalgias,
arthralgias, neuropathy, low leukocytes and platelets, elevated erythrocyte sedimentation rate, positive
antinuclear antibodies (ANA), anti-
double-stranded DNA (dsDNA),
antineutrophil cytoplasmic antibodies (
ANCA), low
complement, and
rheumatoid factor were more frequent in SCNSV (p < 0.05). In cerebrospinal fluid,
pleocytosis and
proteins were higher in PCNSV (p < 0.05). Periventricular and subcortical hyperintense lesions were observed in cranial magnetic resonance imaging in both
vasculitides. Cerebral angiography and angioresonance showed narrowing of vasculature in all patients in both groups. CB showed
gliosis and lymphocytic infiltration within and around the walls in four patients and granulomatous infiltration in the other patients.
After treatment, the Kaplan-Meier survival curve showed a higher relapse-free survival in PCNSV (p < 0.05).
Neurological manifestations and neuroimaging findings were similar in both groups of
vasculitides, but general symptoms, joint, musculoskeletal, and
peripheral neuropathy were preponderant in SCNSV.
After treatment with IV-CYC and GCS, patients with PCNSV had a higher relapse-free survival than those with SCNSV.