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Central nervous system angiitis: a series of 31 patients.
MedLine Citation:
PMID:  24096639     Owner:  NLM     Status:  Publisher    
Central nervous system (CNS) angiitis is a rare inflammatory disorder. To date, clinical data are lacking and treatment remains a matter of debate. The aim of this study is to analyse the main characteristics, response to therapy and outcome of 32 patients with CNS angiitis. Single-centre retrospective study in a tertiary centre was made. Diagnosis of CNS angiitis was made by cerebral angiography and/or magnetic resonance angiography and/or CNS biopsy. The main features and outcomes of primary and secondary CNS angiitis were compared and predictive factors of a favourable outcome were searched. Thirty-one patients (median age 45 Q1-Q3 37-54) sex ratio F/M 2.1) were included. Main clinical features were hemiparesis (35.5 %) and headache (29 %). The median CSF protein level was 0.64(0.52-0.81) g/L and was superior to 1 g/L in six cases. CNS magnetic resonance (MR) imaging findings were most frequently ischemic (96.8 %), bilateral (83.9 %), multiple (87.1 %) and supratentorial (96.8 %). The MR angiography was abnormal in all cases. Among the 31 patients in the study, 19 (61.3 %) were diagnosed with primary CNS angiitis. Systemic lupus erythematosus (n = 6) and vasculitis (n = 4) were the most frequent aetiologies of secondary CNS angiitis. No difference was evidenced between primary and secondary CNS angiitis. Steroids were administered in 79.2 % of treated patients and combined with immunosuppressants in 79.2 % of cases. Eight cases of CNS angiitis relapse were noted. CNS angiitis remains a severe illness. Treatment often associated steroids and immunosuppressants, and diagnosis delay is significantly associated with a poorer prognosis.
Guillaume Geri; David Saadoun; Rémy Guillevin; Sophie Crozier; Catherine Lubetzki; Karima Mokhtari; Zahir Amoura; Bertrand Wechsler; Du Le Boutin; Nathalie Costedoat-Chalumeau; Yves Samson; Patrice Cacoub
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-10-6
Journal Detail:
Title:  Clinical rheumatology     Volume:  -     ISSN:  1434-9949     ISO Abbreviation:  Clin. Rheumatol.     Publication Date:  2013 Oct 
Date Detail:
Created Date:  2013-10-7     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8211469     Medline TA:  Clin Rheumatol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Service de Médecine Interne II, AP-HP, Hôpital Pitié-Salpêtrière, Paris, 75013, France.
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