Primary and secondary central nervous system vasculitis: clinical manifestations, laboratory findings, neuroimaging, and treatment analysis

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 glucocorticoste...

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Bibliographic Details
Published in:Clinical rheumatology 2015-04, Vol.34 (4), p.729-738
Main Authors: Vera-Lastra, Olga, Sepúlveda-Delgado, Jesús, Cruz-Domínguez, María del Pilar, Medina, Gabriela, Casarrubias-Ramírez, Moisés, Molina-Carrión, Luis E., Pineda-Galindo, Luis F., Olvera-Acevedo, Arturo, Hernández-Gonzalez, Claudia, Jara, Luis J.
Format: Article
Language:English
Subjects:
Adult
Biopsy
Cerebral Angiography
Cyclophosphamide - administration & dosage
Disease-Free Survival
Female
Follow-Up Studies
Headache
Humans
Kaplan-Meier Estimate
Magnetic Resonance Imaging
Male
Medicine
Medicine & Public Health
Methylprednisolone - administration & dosage
Middle Aged
Neuroimaging
Original Article
Prednisone - administration & dosage
Recurrence
Rheumatology
Vasculitis - diagnosis
Vasculitis, Central Nervous System - diagnosis
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Summary: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  
ISSN:0770-3198
1434-9949
DOI:10.1007/s10067-014-2831-8