Background Primary central nervous system vasculitis (PCNSV) is a challenging clinical problem due to itsnon- specific signs and symptoms, inaccessibility of the central nervous system (CNS) tissue for pathologic examination, lack of efficient non-invasive diagnostic tests and the relative rarity of it presentation. Secondary central nervous system vasculitis (SCNSV) occurs in association with autoimmune rheumatic diseases (ARD), infections, lymphoproliferative diseases, drug abuse, and systemic vasculitis.
Objectives To compare the initial clinical, laboratory and imaging features in primary central nervous system vaculitis (PCNSV) versus secondary central nervous system vasculitis (SCNSV) and follow-up after treatment with intravenous cyclophosphamide (IV CYC) plus methylprednisolone (MP).
Methods Neurological (focal and non-focal manifestations), laboratory (cerebrospinal fluid and immunological tests) and neuroimaging findings were analyzed in PCNSV and SCNSV patients. Both groups received at onset MPplus IV CYC during 6 months, followed by bimonthly IV CYC plus oral glucocorticosteroids (CGS) for 12 months. All the patients were followed for 36 months.
Results Thirty patients were included (12 PCNSV and 18 SCNSV). Focal and non- focal manifestations were similar in both groups (p=NS); headache being the most frequent manifestation in both groups. Fatigue, myalgias, arthralgias, neuropathy low leukocytes and platelets, elevatederythrocyte sedimentation rate, positive ANA, anti ds-DNA, ANCA, low complement, and rheumatoid factor were more frequent in SCNSV (p<0.05). In cerebrospinal fluid (CSF) pleocytosis and proteins were higher in PCNSV (p<0.05). Periventricular and subcortical hyperintense lesions were observed incranial magnetic resonance imaging in both vasculitides. Cerebral angiography and angioresonance showed narrowing of vasculature in both groups. After treatment, Kaplan-Meier survival curve showed higher relapse free survival in PCNSV (p<0.05).
Conclusions There are significant differences in the clinical manifestations, laboratory, and CSF findings between PCNSV and SCNSV. After treatment with IV CYC and GCS patients with PCNSV had higher relapse-free survival than SCNSV.
Disclosure of Interest None Declared