Wegener’s granulomatosis effectively treated with rituximab: a case study

Abstract

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.