Bilateral Facial Palsy in Rapidly Progressive Course of Wegener’s Granulomatosis: A Case Report

Introduction. Wegener’s granulomatosis belongs to a group of systemic vasculitis diseases, which is characterized by necrotizing vasculitis and presence of granulomas. In a lot of cases, the first symptoms of the disease are observed in the head and neck region, but the bilateral facial nerve palsy occurs very rarely. Objective. The objective of our report was to describe the unusual course of Wegener’s granulomatosis with the bilateral facial nerve paralysis, which subsided after application of steroids and immunosuppressive therapy in combination with surgical treatment. Results and Conclusions. Hearing loss may precede other symptoms in Wegener’s granulomatosis. Ear pain and otorrhea may suggest the diagnosis of bacterial purulent otitis media and delay the proper diagnosis. In the presented case, considering the clinical course, it was necessary to apply both pharmacological and surgical treatments. 1. Introduction Wegenr’s granulomatosis (WG) is a rare systemic autoimmune disease. It is estimated that in Europe there are 25–150 individuals per million suffering from WG and 5–10 new cases per million annually [1]. The increased incidence is recorded in the Scandinavian countries, compared to the countries of southern Europe. Using the “Northsouth” factor, in Norway an average of 12 new cases per million inhabitants per year was recorded and in Spain four times less 3 per million [2]. The new nomenclature defines this disease as granulomatosis with polyangiitis—GPA [3]. This name better reflects the nature of this pathology. granulomatosis refers to necrotizing granulomatous vasculitis, and polyangiitis concerns mainly small-diameter vessels belonging to the group of diseases called microscopic polyangiitis MPA [4]. The disease occurs in both young and adults, with no gender predilection. The average age at diagnosis is 40 [5, 6]. Changes are located typically in both upper and lower respiratory tract and in kidneys (rapidly progressive glomerulonephritis). Depending on the number of affected organs, there are two forms of the disease, isolated form and generalized form. Any organ can be affected. Occasionally, it can be the nervous system, gastrointestinal tract, heart, eyeball, osteoarticular system, and mammary gland [1, 7]. Current treatment regimens based on combination therapy with steroids and immunosuppressive drugs (cyclophosphamide, methotrexate) allow for remission in 70–80% of cases [2, 8, 9]. Lack of treatment might be fatal in 80–82% patients in the first year after diagnosis [9, 10]. Patients with WG have an average survival of 5