Rituximab in the treatment of Wegener’s granulomatosis non-responsive to standard therapy


Authors: P. Němec 1;  J. Vlček 1;  V. Postránecká 2;  A. Krpenský 3;  M. Souček 1
Authors‘ workplace: II. interní klinika, FN u sv. Anny v Brně a Lékařská fakulta Masarykovy univerzity, Brno 1;  Klinika zobrazovacích metod, FN u sv. Anny v Brně a Lékařská fakulta Masarykovy univerzity, Brno 2;  I. patologicko-anatomický ústav, FN u sv. Anny v Brně a Lékařská fakulta Masarykovy univerzity, Brno 3
Published in: Čes. Revmatol., 17, 2009, No. 1, p. 51-61.
Category: Case Report

Overview

Wegener’s granulomatosis (WG) is a severe systemic disease characterised by the development of granulomatous inflammation, tissue necrosis and vasculitis of small- and medium-sized vessels. The pulmonary involvement with a risk of the development of alveolar haemorrhage, and renal involvement characterised by necrotizing glomerulonephritis with crescent formation endangering the patient by the development of rapidly progressive renal failure, belong to the most severe organ manifestations threatening the patient’s life. The standard induction therapy leads to remission in more than 90% of patients. This disease is marked by a substantial tendency to flare. Frequent flares of WG non-responsive to standard treatment can be alternatively managed by so called “rescue therapy“. We present a case report of a patient with WG non-responsive to standard induction therapy, treated with rituximab. Rituximab is a chimeric monoclonal antibody specific for cell membrane antigen CD20 of mature B-lymphocytes, administration of which leads to a depletion of peripheral B-lymphocytes. Results of some earlier published studies suggest a decrease of ANCA production and a possible induction of remission after the administration of rituximab to patients with ANCA associated vasculitis.

Key words:
rituximab, therapy, Wegener’s granulomatosis, inflammation


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