title: Central nervous system involvement of granulomatosis with polyangiitis: clinical-radiological presentation distinguishes different outcomes.
abstract: OBJECTIVE
  The aim of this study was to describe the presentation and outcomes of patients with granulomatosis with polyangiitis (GPA) presenting with CNS involvement.
  
  
  METHODS
  Patients were included in this nationwide retrospective study if they had GPA according to ACR criteria and/or the European Medicines Agency algorithm and CNS involvement.
  
  
  RESULTS
  Thirty-five patients were included in the study. CNS involvement was observed in 51% of patients at GPA diagnosis. Headache (66%) was the main symptom, followed by sensory (43%) and motor impairment (31%). CNS involvement was characterized by pachymeningitis in 20, cerebral ischaemic lesions in 15 and haemorrhagic lesions in 2, with hypophyseal involvement in 2 patients. According to the clinical-radiological presentation, we distinguished granulomatous (G-CNS) and vasculitic (V-CNS) phenotypes. G-CNS patients more frequently had headaches, while V-CNS patients more frequently had motor impairment and renal involvement. Induction therapy produced clinical responses in 86% of patients. Baseline modified Rankin scale was higher for V-CNS than G-CNS patients (3 vs 2, P = 0.002). Initial spinal cord pachymeningitis was significantly associated with the need for a new induction regimen for relapsing/refractory disease (P = 0.01). Long-term neurological sequelae were noted in 51% of patients, including 35% with G-CNS and 69% with V-CNS (P = 0.08). Neurological sequelae were mainly noted in cases of spinal cord pachymeningitis (100%) and ischaemic or haemorrhagic lesions (73%).
  
  
  CONCLUSIONS
  The clinical-radiological phenotype distinguished different long-term outcomes in patients with GPA and CNS involvement. Long-term neurological sequelae persisted in half of patients, mainly those with spinal cord pachymeningitis and vasculitic lesions.
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Cochin Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France, Department of Internal Medicine, Hospital Ramon y Cajal, Madrid, Spain, Department of Internal Medicine, Centre Hospitalier Universitaire, Nancy, Department of Internal Medicine, Centre Hospitalier Universitaire, Montpellier, Department of Internal Medicine, HP Metz Site Belle Isle, Metz, France, Department of internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain, Department of Internal Medicine, Centre Hospitalier, Vannes and Department of Rheumatology, Bicêtre Hospital, AP-HP, Université Paris-Sud, Le Kremlin-Bicêtre, France. Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Cochin Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France, Department of Internal Medicine, Hospital Ramon y Cajal, Madrid, Spain, Department of Internal Medicine, Centre Hospitalier Universitaire, Nancy, Department of Internal Medicine, Centre Hospitalier Universitaire, Montpellier, Department of Internal Medicine, HP Metz Site Belle Isle, Metz, France, Department of internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain, Department of Internal Medicine, Centre Hospitalier, Vannes and Department of Rheumatology, Bicêtre Hospital, AP-HP, Université Paris-Sud, Le Kremlin-Bicêtre, France.
authors.forename: Gonzalo
authors.initials: G
authors.lastname: De Luna
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Cochin Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France, Department of Internal Medicine, Hospital Ramon y Cajal, Madrid, Spain, Department of Internal Medicine, Centre Hospitalier Universitaire, Nancy, Department of Internal Medicine, Centre Hospitalier Universitaire, Montpellier, Department of Internal Medicine, HP Metz Site Belle Isle, Metz, France, Department of internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain, Department of Internal Medicine, Centre Hospitalier, Vannes and Department of Rheumatology, Bicêtre Hospital, AP-HP, Université Paris-Sud, Le Kremlin-Bicêtre, France. benjamin.terrier@cch.aphp.fr.
authors.forename: Benjamin
authors.initials: B
authors.lastname: Terrier
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Cochin Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France, Department of Internal Medicine, Hospital Ramon y Cajal, Madrid, Spain, Department of Internal Medicine, Centre Hospitalier Universitaire, Nancy, Department of Internal Medicine, Centre Hospitalier Universitaire, Montpellier, Department of Internal Medicine, HP Metz Site Belle Isle, Metz, France, Department of internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain, Department of Internal Medicine, Centre Hospitalier, Vannes and Department of Rheumatology, Bicêtre Hospital, AP-HP, Université Paris-Sud, Le Kremlin-Bicêtre, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Kaminsky
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Cochin Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France, Department of Internal Medicine, Hospital Ramon y Cajal, Madrid, Spain, Department of Internal Medicine, Centre Hospitalier Universitaire, Nancy, Department of Internal Medicine, Centre Hospitalier Universitaire, Montpellier, Department of Internal Medicine, HP Metz Site Belle Isle, Metz, France, Department of internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain, Department of Internal Medicine, Centre Hospitalier, Vannes and Department of Rheumatology, Bicêtre Hospital, AP-HP, Université Paris-Sud, Le Kremlin-Bicêtre, France.
authors.forename: Alain
authors.initials: A
authors.lastname: Le Quellec
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Cochin Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France, Department of Internal Medicine, Hospital Ramon y Cajal, Madrid, Spain, Department of Internal Medicine, Centre Hospitalier Universitaire, Nancy, Department of Internal Medicine, Centre Hospitalier Universitaire, Montpellier, Department of Internal Medicine, HP Metz Site Belle Isle, Metz, France, Department of internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain, Department of Internal Medicine, Centre Hospitalier, Vannes and Department of Rheumatology, Bicêtre Hospital, AP-HP, Université Paris-Sud, Le Kremlin-Bicêtre, France.
authors.forename: François
authors.initials: F
authors.lastname: Maurier
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Cochin Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France, Department of Internal Medicine, Hospital Ramon y Cajal, Madrid, Spain, Department of Internal Medicine, Centre Hospitalier Universitaire, Nancy, Department of Internal Medicine, Centre Hospitalier Universitaire, Montpellier, Department of Internal Medicine, HP Metz Site Belle Isle, Metz, France, Department of internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain, Department of Internal Medicine, Centre Hospitalier, Vannes and Department of Rheumatology, Bicêtre Hospital, AP-HP, Université Paris-Sud, Le Kremlin-Bicêtre, France.
authors.forename: Roser
authors.initials: R
authors.lastname: Solans
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Cochin Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France, Department of Internal Medicine, Hospital Ramon y Cajal, Madrid, Spain, Department of Internal Medicine, Centre Hospitalier Universitaire, Nancy, Department of Internal Medicine, Centre Hospitalier Universitaire, Montpellier, Department of Internal Medicine, HP Metz Site Belle Isle, Metz, France, Department of internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain, Department of Internal Medicine, Centre Hospitalier, Vannes and Department of Rheumatology, Bicêtre Hospital, AP-HP, Université Paris-Sud, Le Kremlin-Bicêtre, France.
authors.forename: Pascal
authors.initials: P
authors.lastname: Godmer
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Cochin Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France, Department of Internal Medicine, Hospital Ramon y Cajal, Madrid, Spain, Department of Internal Medicine, Centre Hospitalier Universitaire, Nancy, Department of Internal Medicine, Centre Hospitalier Universitaire, Montpellier, Department of Internal Medicine, HP Metz Site Belle Isle, Metz, France, Department of internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain, Department of Internal Medicine, Centre Hospitalier, Vannes and Department of Rheumatology, Bicêtre Hospital, AP-HP, Université Paris-Sud, Le Kremlin-Bicêtre, France.
authors.forename: Nathalie
authors.initials: N
authors.lastname: Costedoat-Chalumeau
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Cochin Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France, Department of Internal Medicine, Hospital Ramon y Cajal, Madrid, Spain, Department of Internal Medicine, Centre Hospitalier Universitaire, Nancy, Department of Internal Medicine, Centre Hospitalier Universitaire, Montpellier, Department of Internal Medicine, HP Metz Site Belle Isle, Metz, France, Department of internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain, Department of Internal Medicine, Centre Hospitalier, Vannes and Department of Rheumatology, Bicêtre Hospital, AP-HP, Université Paris-Sud, Le Kremlin-Bicêtre, France.
authors.forename: Raphaèle
authors.initials: R
authors.lastname: Seror
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Cochin Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France, Department of Internal Medicine, Hospital Ramon y Cajal, Madrid, Spain, Department of Internal Medicine, Centre Hospitalier Universitaire, Nancy, Department of Internal Medicine, Centre Hospitalier Universitaire, Montpellier, Department of Internal Medicine, HP Metz Site Belle Isle, Metz, France, Department of internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain, Department of Internal Medicine, Centre Hospitalier, Vannes and Department of Rheumatology, Bicêtre Hospital, AP-HP, Université Paris-Sud, Le Kremlin-Bicêtre, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Charles
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Cochin Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France, Department of Internal Medicine, Hospital Ramon y Cajal, Madrid, Spain, Department of Internal Medicine, Centre Hospitalier Universitaire, Nancy, Department of Internal Medicine, Centre Hospitalier Universitaire, Montpellier, Department of Internal Medicine, HP Metz Site Belle Isle, Metz, France, Department of internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain, Department of Internal Medicine, Centre Hospitalier, Vannes and Department of Rheumatology, Bicêtre Hospital, AP-HP, Université Paris-Sud, Le Kremlin-Bicêtre, France.
authors.forename: Pascal
authors.initials: P
authors.lastname: Cohen
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Cochin Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France, Department of Internal Medicine, Hospital Ramon y Cajal, Madrid, Spain, Department of Internal Medicine, Centre Hospitalier Universitaire, Nancy, Department of Internal Medicine, Centre Hospitalier Universitaire, Montpellier, Department of Internal Medicine, HP Metz Site Belle Isle, Metz, France, Department of internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain, Department of Internal Medicine, Centre Hospitalier, Vannes and Department of Rheumatology, Bicêtre Hospital, AP-HP, Université Paris-Sud, Le Kremlin-Bicêtre, France.
authors.forename: Xavier
authors.initials: X
authors.lastname: Puéchal
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Cochin Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France, Department of Internal Medicine, Hospital Ramon y Cajal, Madrid, Spain, Department of Internal Medicine, Centre Hospitalier Universitaire, Nancy, Department of Internal Medicine, Centre Hospitalier Universitaire, Montpellier, Department of Internal Medicine, HP Metz Site Belle Isle, Metz, France, Department of internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain, Department of Internal Medicine, Centre Hospitalier, Vannes and Department of Rheumatology, Bicêtre Hospital, AP-HP, Université Paris-Sud, Le Kremlin-Bicêtre, France.
authors.forename: Luc
authors.initials: L
authors.lastname: Mouthon
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Cochin Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France, Department of Internal Medicine, Hospital Ramon y Cajal, Madrid, Spain, Department of Internal Medicine, Centre Hospitalier Universitaire, Nancy, Department of Internal Medicine, Centre Hospitalier Universitaire, Montpellier, Department of Internal Medicine, HP Metz Site Belle Isle, Metz, France, Department of internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain, Department of Internal Medicine, Centre Hospitalier, Vannes and Department of Rheumatology, Bicêtre Hospital, AP-HP, Université Paris-Sud, Le Kremlin-Bicêtre, France.
authors.forename: Loic
authors.initials: L
authors.lastname: Guillevin
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D002545:Brain Ischemia / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000188:drug therapy; D002493:Central Nervous System Diseases / Q000175:diagnosis* / Q000000981:diagnostic imaging* / Q000188:drug therapy; D002648:Child; D002675:Child, Preschool; D005260:Female; D005602:France; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000175:diagnosis* / Q000453:epidemiology; D006261:Headache / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000188:drug therapy; D006801:Humans; D008297:Male; D008581:Meningitis / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000188:drug therapy; D008875:Middle Aged; D010641:Phenotype; D015995:Prevalence; D011379:Prognosis; D011859:Radiography; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones
keywords: ANCA; central nervous system; cerebral vasculitis; granulomatosis with polyangiitis; pachymeningitis