Successful rituximab treatment in a patient with ANCA-negative granulomatosis with polyangitis: A case report.

A 68-year-old woman was referred to our hospital for further evaluation of fever, nasal congestion, deafness, and multiple pulmonary nodules refractory to antibiotic use. Despite negative findings of antineutrophil cytoplasmic antibodies, she was diagnosed with granulomatosis with polyangiitis based on the analysis of biopsy specimens of pulmonary nodules. The administrations of oral prednisolone and six intravenous cyclophosphamide (IVCY) resulted in the prompt relief of symptoms and disappearance of pulmonary nodules. However, 3 months after the completion of IVCY therapy, nasal congestion and deafness flared up with an increase in the C-reactive protein level; a repeat computed tomography revealed a left lung nodule. Consequently, she underwent remission induction and maintenance therapy with rituximab (RTX), which resulted in the symptomatic improvement and disappearance of pulmonary nodules after 6 months. The patient remained in remission thereafter. Therefore, RTX may be an effective therapeutic option even in the absence of detectable autoantibodies.