[Efficacy of rituximab in refractory Wegener's granulomatosis with central nervous system involvement]

Wegener's granulomatosis (WG) is characterized by inflammatory granulomas with necrotizing vasculitis of small and medium-sized blood vessels usually associated with the presence of circulating antineutrophil cytoplasmic antibodies (c-ANCA). The disease predominantly affects the upper and lower airways and kidneys. Besides this classic triad of affected organs, less frequent manifestations of WG include involvement of the skin, central nervous system (CNS), and eyeballs (often with proptosis), as well as otitis with progressive hearing loss. The frequency of CNS involvement according to various researchers lies between 4% and 11% of WG cases. Dura mater infiltrates, cranial nerve pathology, and vasculitis are the most frequent CNS lesions. They concur with clinical symptoms such as paresthesia, motor function impairment, aphasia, and mood disorders. Three major mechanisms have been implicated in CNS involvement accompanying WG: contiguous growth of granuloma from extracranial sites (e.g., paranasal sinuses), intracranial granuloma (tumor) formation, and vasculitis. The severe form of WG is usually treated with a combination of cyclophosphamide and corticosteroids. In refractory cases, rituximab known to bind to CD20 expressed on B-cells, should be considered.

We present the case of a 38-year-old woman with a severe form of WG that was refractory to standard therapy. The disease was generalized from its onset and after 6 months. Cranial MRI revealed infiltration of dura mater at the sella turcica near the left cavernous sinus and infiltration in the left orbital apex surrounding the optic nerve. These lesions caused left-side oculomotor nerve paresis with blindness and severe ipsilateral headaches. Rituximab produced considerable regression of MRI lesions and disappearance of the majority of clinical symptoms.