Central nervous system involvement in Wegener granulomatosis.
Wegener granulomatosis (WG) is an antineutrophil cytoplasmic antibody (ANCA)-associated granulomatous vasculitis of small and medium-sized vessels. This vasculitis involves mainly the upper and lower respiratory tracts and kidneys, although WG may affect any organ. Central nervous system (CNS) involvement is an uncommon manifestation of WG, reported in 7%-11% of patients. Three major mechanisms have been incriminated as causing CNS disease in WG: contiguous invasion of granuloma from extracranial sites, remote intracranial granuloma, and CNS vasculitis. Herein we describe 6 patients with WG-related CNS involvement, 2 of whom had chronic hypertrophic pachymeningitis, 3 with pituitary involvement, and 1 with cerebral vasculitis. CNS involvement was present at disease onset in 2 patients and occurred 5-18 years after WG diagnosis in the remaining 4. Based on these observations and a review of the literature, we discuss the pathogenic mechanisms, clinical features, imaging findings, treatment, and outcome of meningeal, pituitary, and vascular involvement, with an emphasis on differential diagnoses, prognosis, and therapeutic management of WG-related CNS involvement.
Seror
Raphaèle
R
From Department of Internal Medicine, Hôpital Cochin, Université René-Descartes Paris 5, Assistance Publique-Hôpitaux de Paris, Paris, France.
Mahr
Alfred
A
Ramanoelina
Jacky
J
Pagnoux
Christian
C
Cohen
Pascal
P
Guillevin
Loïc
L
D000328:Adult; D002493:Central Nervous System Diseases / Q000209:etiology* / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged