Gonzalo De Luna1, Benjamin Terrier2, Pierre Kaminsky3, Alain Le Quellec3, François Maurier3, Roser Solans3, Pascal Godmer3, Nathalie Costedoat-Chalumeau3, Raphaèle Seror3, Pierre Charles3, Pascal Cohen3, Xavier Puéchal3, Luc Mouthon3, Loic Guillevin3. 1. 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. 2. 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. 3. 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.
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.
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.