Manifestation of eosinophilic granulomatosis with polyangiitis in the head and neck area over time taking systemic disease activity into consideration

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is arare granulomatous vasculitis of the small and medium sized blood vessels of unknown etiology. Aregular involvement of the head and neck area is known. Since it is not yet known whether the inflammatory processes in the head and neck area are parallel to the systemic disease activity, the course of systemic and local disease activity was investigated in acomparative study. Between 1990 and 2010 the disease progression of 25patients with atotal of 90consultations and an average observation period of 7.02 years was recorded. The anamnesis, clinical observation and testing were performed in order to describe ear nose and throat (ENT) manifestations. Moreover, systemic data, such as the Birmingham Vasculitis Activity Score(BVAS), the Vasculitis Damage Index (VDI) and the serological parameters C‑reactive protein (CRP), eosinophilic cationic protein (ECP) and absolute eosinophil count (EOS) were collected. In order to define the activity in the head and neck area the existing manifestations were summarized and statistically compared to the systemic data using Spearman's rank correlation coefficient. Overall the statistical analysis showed avery weak correlation between the disease activity in the ENT area and systemic disease activity, which is not considered as significant. Comparing activity in the nose region to systemic activity, significant values were found in 22.9% of all consultations. Comparing activity in the ear region to systemic activity, 11.8% of all consultations showed significant values. The results indicate that the disease activity in the head and neck area is independent of the systemic activity. Moreover, they point out apotential independent autoimmune inflammatory process in the mentioned regions; therefore, treatment should include long-term follow-up and an interdisciplinary management.