Central nervous system involvement of granulomatosis with polyangiitis: clinical-radiological presentation distinguishes different outcomes
Open Access
- 3 September 2014
- journal article
- research article
- Published by Oxford University Press (OUP) in Rheumatology
- Vol. 54 (3), 424-432
- https://doi.org/10.1093/rheumatology/keu336
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%). Conclusion. 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.Keywords
This publication has 25 references indexed in Scilit:
- Exceptional osseous and meningeal spinal localization of ANCA-associated granulomatous vasculitis with hypertrophic spinal pachymeningitisZeitschrift für Neurologie, 2011
- Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studiesAnnals Of The Rheumatic Diseases, 2006
- Central Nervous System Involvement in Wegener GranulomatosisMedicine, 2006
- Incidence et présentation des manifestations neurologiques centrales au cours de la granulomatose de Wegener : analyse monocentrique d’une série de 14 maladesLa Revue de Médecine Interne, 2004
- Standardized Neurologic Evaluations of 128 Patients With Wegener GranulomatosisArchives of Neurology, 2001
- An interdisciplinary approach to the care of patients with Wegener's granulomatosis: Long-term outcome in 155 patientsArthritis & Rheumatism, 2000
- Development and initial validation of the vasculitis damage index for the standardized clinical assessment of damage in the systemic vasculitidesArthritis & Rheumatism, 1997
- Neurological involvement in Wegener's granulomatosis: An analysis of 324 consecutive patients at the Mayo ClinicAnnals of Neurology, 1993
- Wegener Granulomatosis: An Analysis of 158 PatientsAnnals of Internal Medicine, 1992
- Wegener's Granulomatosis: Prospective Clinical and Therapeutic Experience With 85 Patients for 21 YearsAnnals of Internal Medicine, 1983