[Efficacy of rituximab in refractory Wegener's granulomatosis with central nervous system involvement]. / Skutecznosc rytuksymabu w leczeniu opornej ziarniniakowatosci Wegenera z zajefciem osrodkowego ukladu nerwowego.

INTRODUCTION: 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. CASE REPORT: 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.

Wegener's granulomatosis effectively treated with rituximab: a case study.

Wegener's granulomatosis (WG) is a granulomatous disorder associated with systemic necrotizing vasculitis. Wegener's granulomatosis predominantly involves the upper airways, lung and kidneys. The disease is often associated with cytoplasmic antineutrophil cytoplasmic antibodies (cANCA). B lymphocytes are potential cANCA producers and there is an evident correlation between cANCA titre, severity of the disease and response to treatment. Wegener's granulomatosis usually begins with symptoms limited mostly to the upper and/or lower respiratory tracts and may transform into the generalized phase, characterized by systemic necrotizing vasculitis. If left untreated, it can turn fulminant with poor prognosis. The severe form of the disease is usually treated with a combination of cyclophosphamide and corticosteroids. In refractory cases, rituximab that binds to CD20 expressed on B-cells should be considered. We presented a case of a 38-year-old woman with severe form of WG, refractory to standard therapy. Despite the standard treatment with cyclophosphamide and corticosteroids and the addition of infliximab with methotrexate, progression of the disease was observed. Exacerbation affected mainly the lungs and caused the gradual destruction of pulmonary tissue and development of respiratory insufficiency. Rituximab (500 mg) was given intravenously every week in four infusions, causing a partial remission of WG and the arrest of lung deterioration. The following administration of 500 mg was given every two weeks, which induced the remission of WG and enabled the patient to return to her normal activity and work. Such treatment appeared to be successful and prevented severe pulmonary involvement.