title: Granulomatosis polyangiitis associated with meningeal involvement: response to rituximab therapy after failure of cyclophosphamide.
abstract: Wegener's granulomatosis or granulomatosis polyangiitis associated (GPA) is a granulomatous inflammation of the upper and lower respiratory tracts associated with necrotising vasculitis of small and medium-sized blood vessels and, frequently, necrotising glomerulonephritis. We describe the case of a 37 year old female patient presenting with upper respiratory tract involvement, chronic rhinosinusitis with green secretions, and bilateral hypoacusia. Ten months later, she suffered occipital headache and two episodes of lipothymia associated with nausea, photophobia, faintness with visual blurring. Magnetic resonance imaging (MRI) revealed aseptic meningitis. The patient was treated with steroids and cyclophosphamide without any effect on the neurological symptoms which, however, greatly improved after subsequent treatment with rituximab as confirmed by means of cerebral MRI. Rituximab is an optimal means of treating cyclophosphamide-resistant patients with GPA associated with meningeal involvement.
authors.affiliation: Rheumatology Unit, San Giovanni di Dio Hospital, Florence, Italy. maurizio.benucci@asf.toscana.it
authors.forename: M
authors.initials: M
authors.lastname: Benucci
authors.forename: F
authors.initials: F
authors.lastname: Li Gobbi
authors.forename: P
authors.initials: P
authors.lastname: Panconesi
authors.forename: M
authors.initials: M
authors.lastname: Manfredi
authors.forename: P
authors.initials: P
authors.lastname: Sarzi-Puttini
authors.forename: F
authors.initials: F
authors.lastname: Atzeni
mesh_terms: D000328:Adult; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000188:drug therapy*; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008581:Meningitis / Q000188:drug therapy* / Q000209:etiology*; D012074:Remission Induction; D000069283:Rituximab; D017211:Treatment Failure
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D000069283:Rituximab; D003520:Cyclophosphamide