Eosinophilic granulomatosis with polyangiitis (EGPA) is an anti-neutrophil cytoplasmic autoantibody-related vasculitis, that is characterized by systemic vasculitis with marked eosinophilia. Rhinosinusitis is a major manifestation of EGPA, and otorhinolaryngologists treating patients with chronic eosinophilic rhinosinusitis (ECRS) should be aware of the risk of development of EGPA in these patients; however, few reports have addressed the disease characteristics and essential patient guidance. Herein, we present the case of a patient with EGPA who developed postoperative cerebral infarction after surgical treatment for ECRS.
A 60-year-old male patient was evaluated for nasal obstruction and smell disturbance. Physical examination showed bilateral polyps in the nasal cavities. Computed tomography revealed bilateral pansinusitis, predominantly in the ethmoid sinus. Nasal polyp biopsy revealed eosinophilic infiltration. Based on the diagnosis of ECRS, the patient was scheduled for bilateral endoscopic sinus surgery. In addition, preoperative evaluation revealed bronchial asthma, and the patient was started on inhaled bronchodilator/steroid therapy.
During the postoperative course, the patient frequently experienced exacerbation of sinus mucosal edema, and received multiple short courses of oral steroids. The patient sought medical consultation 25 months after the surgery, complaining of a sensation of coldness in both the lower limbs and a four-day history of numbness in both hands and weakness in all limbs. High-resolution magnetic resonance imaging of the brain showed infarction in both the putamen and the thalamus. Blood examination showed a markedly increased eosinophil count (48%), and the patient was diagnosed as having EGPA. Remission was achieved with pulse steroid therapy and immunosuppressants, but the patient was unfortunately left with the sequelae of hemiplegia and dysarthria.
Patients with ECRS should be informed of the risk of development of EGPA and be educated about specific vasculitis symptoms, so as to facilitate early diagnosis. The risk of development of EGPA is higher in patients with peripheral blood differential eosinophil counts in excess of 10% on preoperative examination for ECRS and those showing recurrent inflammation of the sinus mucosa postoperatively. In such cases, close monitoring of the peripheral blood eosinophil counts is critical throughout the follow-up period.
