Central nervous system angiitis: a series of 31 patients

Autor:	David Saadoun, Du Le Thi Huong Boutin, Nathalie Costedoat-Chalumeau, Sophie Crozier, Karima Mokhtari, Patrice Cacoub, Yves Samson, Bertrand Wechsler, Guillaume Geri, Catherine Lubetzki, Zahir Amoura, Rémy Guillevin
Přispěvatelé:	Service de Réanimation polyvalente, Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Cochin [AP-HP]-Université Paris Descartes - Paris 5 (UPD5), Immunologie - Immunopathologie - Immunothérapie (I3), Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Pierre et Marie Curie - Paris 6 (UPMC), Service de Radiologie [Milétrie], Centre hospitalier universitaire de Poitiers (CHU Poitiers), Service des Urgences Cérébro-Vasculaires [Paris], CHU Pitié-Salpêtrière [APHP], Département de Neurologie [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-IFR70-CHU Pitié-Salpêtrière [APHP], Centre de Recherche de l'Institut du Cerveau et de la Moelle épinière (CRICM), Service de Neuropathologie, Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Pitié-Salpêtrière [APHP], Centre d'Immunologie et de Maladies Infectieuses (CIMI), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service de médecine interne 2 [Pitié-Salpétrière], Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Charles Foix - Jean Rostand-CHU Pitié-Salpêtrière [APHP]-Centre de référence des maladies autoimmunes et systémiques rares, Centre de référence des maladies autoimmunes et systémiques rares, Equipe NEMESIS - Centre de Recherches de l'Institut du Cerveau et de la Moelle épinière (NEMESIS-CRICM), Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Pierre et Marie Curie - Paris 6 (UPMC), Colliot, Olivier, Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Cochin [AP-HP]-Université Paris Descartes - Paris 5 ( UPD5 ), Immunologie - Immunopathologie - Immunothérapie ( I3 ), Centre National de la Recherche Scientifique ( CNRS ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Université Pierre et Marie Curie - Paris 6 ( UPMC ), Centre hospitalier universitaire de Poitiers ( CHU Poitiers ), Assistance publique - Hôpitaux de Paris (AP-HP)-IFR70-Hôpital de la Salpétrière, Centre de Recherche de l'Institut du Cerveau et de la Moelle épinière ( CRICM ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ), Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Pitié-Salpêtrière [APHP], Centre d'Immunologie et de Maladies Infectieuses ( CIMI ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Assistance publique - Hôpitaux de Paris (AP-HP)-Hôpital Charles Foix - Jean Rostand-CHU Pitié-Salpêtrière [APHP]-Centre de référence des maladies autoimmunes et systémiques rares, Equipe NEMESIS - Centre de Recherches de l'Institut du Cerveau et de la Moelle épinière ( NEMESIS-CRICM ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ) -Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Descartes - Paris 5 (UPD5), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service des Urgences Cérébro-Vasculaires [CHU Pitié-Salpétriêre], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Neurologie [CHU Pitié-Salpêtrière], IFR70-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)
Jazyk:	angličtina
Rok vydání:	2013
Předmět:	Male Pathology MESH : Retrospective Studies MESH: Cerebral Angiography [SDV.IB.IMA]Life Sciences [q-bio]/Bioengineering/Imaging Biopsy MESH : Magnetic Resonance Angiography [SDV.NEU.NB]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/Neurobiology [INFO.INFO-IM] Computer Science [cs]/Medical Imaging MESH: Magnetic Resonance Angiography [ SPI.SIGNAL ] Engineering Sciences [physics]/Signal and Image processing MESH : Vasculitis Central Nervous System [ INFO.INFO-CV ] Computer Science [cs]/Computer Vision and Pattern Recognition [cs.CV] Gastroenterology Magnetic resonance angiography MESH : Paresis MESH: Biopsy [INFO.INFO-CV] Computer Science [cs]/Computer Vision and Pattern Recognition [cs.CV] Risk Factors MESH: Risk Factors [ INFO.INFO-TI ] Computer Science [cs]/Image Processing MESH : Female MESH : Immunosuppressive Agents CSF albumin [ SDV.IB.IMA ] Life Sciences [q-bio]/Bioengineering/Imaging MESH: Treatment Outcome MESH: Middle Aged medicine.diagnostic_test [ INFO.INFO-IM ] Computer Science [cs]/Medical Imaging Headache General Medicine Middle Aged MESH : Adult MESH : Risk Factors 3. Good health Paresis Treatment Outcome [INFO.INFO-TI] Computer Science [cs]/Image Processing [eess.IV] [ SDV.NEU.NB ] Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/Neurobiology [INFO.INFO-TI]Computer Science [cs]/Image Processing [eess.IV] Female Steroids MESH: Immunosuppressive Agents Vasculitis [SPI.SIGNAL]Engineering Sciences [physics]/Signal and Image processing Immunosuppressive Agents Cerebral angiography Adult medicine.medical_specialty MESH : Male MESH : Treatment Outcome MESH: Paresis Rheumatology Internal medicine medicine MESH : Cerebral Angiography [INFO.INFO-IM]Computer Science [cs]/Medical Imaging Humans MESH : Middle Aged Vasculitis Central Nervous System Retrospective Studies [SPI.SIGNAL] Engineering Sciences [physics]/Signal and Image processing MESH : Headache MESH: Humans business.industry MESH : Humans [SDV.NEU.NB] Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/Neurobiology MESH: Headache [INFO.INFO-CV]Computer Science [cs]/Computer Vision and Pattern Recognition [cs.CV] Retrospective cohort study Magnetic resonance imaging MESH: Adult MESH: Retrospective Studies medicine.disease MESH: Steroids MESH: Male Cerebral Angiography [SDV.IB.IMA] Life Sciences [q-bio]/Bioengineering/Imaging MESH : Biopsy business MESH: Vasculitis Central Nervous System MESH: Female Magnetic Resonance Angiography MESH : Steroids
Zdroj:	Clinical Rheumatology Clinical Rheumatology, Springer Verlag, 2013, 33 (1), pp.105-10 Clinical Rheumatology, 2013, 33 (1), pp.105-10
ISSN:	0770-3198 1434-9949
Popis:	International audience; 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.
Databáze:	OpenAIRE
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