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Abstract
Granulomatosis with polyangiitis (GPA) is a rare anti-neutrophil cytoplasmic antibody associated with systemic disease characterized by granulomas and vasculitis affecting small and medium vessels. Neurological manifestations in GPA are less frequent than classical manifestations, such as lung and kidney involvement, and cranial nerve palsies are much rarer. Cyclophosphamide and glucocorticoids have been conventionally administered as an initial induction immunosuppressive therapy for GPA. However, increasing evidence has demonstrated the efficacy and safety of rituximab, an anti-B cell monoclonal antibody, for the treatment of GPA. Herein, we describe a successful treatment of relapsing GPA with cranial nerve involvement using rituximab in a 56-year-old male patient who was previously treated with cyclophosphamide plus glucocorticoids.
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Figure 1.
Biopsy specimens of the paranasal sinus showing necrotic granulomatous vasculitis without caseous necrosis (A: Hematoxylin and eosin stain, ×40; B: ×200).
Figure 2.
(A) Contrastenhanced T1-weighted image showing an enhancing nodule at the Rt. petro-clival region, entrance site of the right abducens nerve, and Do-rello's canal, suggesting Wegener's granuloma. (B) Contrast enhanced T1-weighted image showing complete resolution of an enhancing nodule after 8 weeks of treatment with rituximab.
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