AB0784 SKULL BASE INFILTRATION AS A MANIFESTATION OF GRANULOMATOSIS WITH POLYANGIITIS

Abstract

BackgroundAlthough upper respiratory symptoms are the most frequent localization of granulomatosis with polyangiitis (GPA) [1], infiltration of the skull base is rare.ObjectivesThe aim of this analysis was the clinical characteristic of GPA patients’ skull base involvement.MethodsThe retrospective analysis was performed on patients diagnosed in tertiary academic referral center (Medical University of Gdansk, Poland) between 1990 and 2022 due to GPA. Medical database of 232 GPA vasculitis patients were retrospectively reviewed, and demographics, serological, and clinical features of the patients presenting skull base infiltration throughout the disease course were recorded. Comparisons of disease characteristics and long-term outcomes were performed between patients with and without this manifestation.ResultsAmong 232 patients (121 males, median age 52,5 years, 87% ANCA positive, 12,5% localized, 87,5% systemic), seven patients presented skull base infiltrations as a manifestation of GPA. It was 4 women and 3 men, aged between 27 and 72. Four patients were cANCA positive, 1 patient had atypical pANCA and 2 patients were ANCA negative. In six of them it was a first manifestation of vasculitis. Clinical characteristic of patients is summarized in table. Statistical analysis showed that patients with skull base involvement and those without this manifestation differed only in the frequency of ocular involvement - in patients with skull base infiltration, ocular involvement occurred in all cases, while among patients without infiltration it occurred in only 34.5 per cent of subjects. In terms of other parameters, there were no statistically significant differences between the groups.ConclusionSkull base infiltrations presenting mainly as cranial nerve palsies is rare presentation of GPA. In most of patients it is one of the first symptoms of vasculitis. It can occur in both ANCA positive and ANCA negative cases.