Experience with successful application of rituximab for the treatment of generalized granulomatosis with polyangiitis (Wegener’s)

Abstract

The paper reports a case of generalized granulomatosis with polyangiitis in a 53 year-old patient with special reference to the difficulties of its diagnostics and successful treatment. The disease started as meningoencephalitis and left-hand hemisinusitis that later developed into pulmonary pathology with multiple foci and large decay cavities in all parts of the lungs. The disease was associated with strongly positive anti-neutrophil cytoplasmic antibodies (ANCAs). Despite treatment with high doses of corticosteroids, pulse therapy, and antibiotic therapy, febrile syndrome persisted, respiratory failure and periodic epileptic seizure progressed. Rituximab therapy in combination with pulse therapy provided a highly efficient tool for the treatment of generalized granulomatosis with polyangiitis.