Background Primary central nervous system vasculitis (PCNSV) is an uncommon condition in which vascular inflammatory lesions are limited to the brain and spinal cord. Diagnostic criteria include a newly acquired neurological deficit that is unexplained by other processes and angiographic or central nervous system (CNS) biopsy features of vasculitis. In contrast secondary central nervous system vasculitis (SCNSV) is vascular inflammation in which CNS involvement is due to diverse causes.
Objectives To investigate the clinical, laboratory, neuroimaging characteristics and treatment in Primary Central Nervous System Vasculitis (PCNSV) versus Secondary Central Nervous System Vasculitis (SCNSV).
Methods We studied twenty female patients with central nervous system vasculitis (CNS) during a period of 3 years in a tertiary level center. The clinical manifestations, laboratory, neuroimaging diagnosis and treatment were analyzed. Patients were divided in PCNSV and SCNSV. Biopsy CNS was performed in 3 patients.
Results We studied 10 PCNSV and 10 SCNSV patients: systemic lupus erythematous 5, systemic sclerosis (SSc) 4, Sjogren syndrome (SS) 1. Mean age in the PCNSV was 26±5 years and SCNSV 47±7years. Clinical manifestation in PCNSV versus SCNSV were: headache 90% vs 60% (NS), fatigue 80% versus 40% (<0.01), paresthesias 90% versus 80% (NS), motor deficit 20%vs 80% (0.01) and ischemic event 40% vs 40% (NS) respectively. The Antinuclear antibodies (ANA’s) were positive 100% in SCNCV vs 20% (<0.001), anti-DNA was present in 100% (<0.01) in SLE patients with SCNSV, hypocomplementemia was 80% in patients with SCNSV and 22% PCNSV (<0.01). Anti Scl-70 were present in 2 patients with SSc, and anti Ro in patients with SS Cerebral spinal fluid (CSF) was negative for infection, neoplastic process and the proteins were elevated in all cases. MRI showed periventricular and subcortical hyperintense lesions in both groups; however, theses lesions were more frequent in SCNSV. Initial treatment was methylprednisolone pulse followed by monthly cyclophosphamide. Maintenance treatment was prednisone (0.5mg/kg/day) alone in 100% of PCNSV cases and in 60% of SCNSV cases, and prednisone plus azathioprine in 40% of SCNSV cases. Recurrence was present in 20% in PCNSV (20%) and in SCNSV (30%).CNS biopsies showed vasculitis.
Conclusions PCNSV had lower frequency of motor deficit, as well as lower ANA’s, and hypocomplementemia in comparison with SCNSV; an increase of CSF proteins was observed in all patients. MRI showed more periventricular and subcortical hyperintense lesions in SCNSV than PCNCV. All patients responded successfully to treatment with steroids plus cyclophosphamide. Early recognition and treatment may reduce poor outcomes.
Salvarini C, Brown RD Jr, Hunder GG. Adult primary central nervous system vasculitis: an update. curr Opin Rheumatol 2012;24:46-52
Salvarini C, Brown RD Jr, Calamia KT, Christianson TJ, Weigand SD, Miller DV, Giannini C, Meschia JF, Huston J3rd, Hunder GG Primary central nervous system vasculitis: analysis of 101 patients. Ann Neurol 2007;62:442-51
Disclosure of Interest None Declared