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Abstract

Response of Chronic, Relapsing Wegener's Granulomatosis with Severe Pulmonary Involvement to Anti-CD20 Monoclonal Antibody (Rituximab)

Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA) are primary systemic small vessel vasculitides with predilection for the respiratory tract and kidneys. Most patients have circulating antineutrophil cytoplasmic antibodies (ANCAs) reacting either with neutrophil proteinase-3 (PR3) or myeloperoxidase (MPO). The combination of glucocorticoids and cylophosphamide is the standart therapy for patients with severe WG or MPO. ANCA-associated vasculitis has a high relapse rate, and some patients do not respond satisfactory to this treatment. Rituximab is a chimeric monoclonal antibody directed against CD20, a cell surface antigen expressed almost exclusively on cells of B-lymphocytes lineage. A rationale for Rituximab in ANCA-associated vasculitis was based on its potential to deplete CD20+ precursors of ANCA-secreting plasma cells. Rituximab is an advanced in the treatment of ANCAassociated vasculitis and has a place in the therapeutic armament as an alternative to Cyclophosphamide or for refractory disease.

We present a case of a young patient with chronic, relapsing ANCA-associated vasculitis (Wegener's granulomatosis) with severe pulmonary involvement in whom the use of Cyclophosphamide failed to control disease activity. Subsequently the patient was treated successfully with Rituximab.

The most frequent complications after the applications of Rituximab are different infections. Some months after the use Rituximab we diagnosed a lobar pneumonia in the patient. The patients with autoimmune diseases are immunosuppressive and are disposed to infections. The aim of the present article is to present the use Rituximab as a future option in the treatment of ANCA-associated vasculitis.


Author(s):

Desislava Kalinova



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