Central nervous system angiitis: a series of 31 patients.

Abstract

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.

• Authors: 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
• Journal: Clinical rheumatology (Clin Rheumatol) Vol. 33 Issue 1 Pg. 105-10 (Jan 2014) ISSN: 1434-9949 [Electronic] Germany
• PMID: 24096639 (Publication Type: Journal Article)

Chemical References

• Immunosuppressive Agents
• Steroids

Topics

• Adult
• Biopsy
• Cerebral Angiography
• Female
• Headache (complications)
• Humans
• Immunosuppressive Agents (administration & dosage)
• Magnetic Resonance Angiography
• Male
• Middle Aged
• Paresis (complications)
• Retrospective Studies
• Risk Factors
• Steroids (administration & dosage)
• Treatment Outcome
• Vasculitis, Central Nervous System (cerebrospinal fluid, diagnosis, pathology)