[Manifestation of eosinophilic granulomatosis with polyangiitis in the head and neck area over time taking systemic disease activity into consideration]. / Manifestation der eosinophilen Granulomatose mit Polyangiitis im Kopf-Hals-Bereich im zeitlichen Verlauf unter Berücksichtigung der systemischen Krankheitsaktivität.

BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare granulomatous vasculitis of the small and medium sized blood vessels of unknown etiology. A regular 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 a comparative study. MATERIAL AND METHODS: Between 1990 and 2010 the disease progression of 25 patients with a total of 90 consultations 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 C­reactive 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. RESULTS: Overall the statistical analysis showed a very 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. CONCLUSION: The results indicate that the disease activity in the head and neck area is independent of the systemic activity. Moreover, they point out a potential independent autoimmune inflammatory process in the mentioned regions; therefore, treatment should include long-term follow-up and an interdisciplinary management.

Manifestation of eosinophilic granulomatosis with polyangiitis in head and neck.

INTRODUCTION: Besides an obvious clinical involvement of the ear, nose and throat (ENT)-region in Eosinophilic Granulomatosis with Polyangiitis (EGPA), systematic data is sparse. Only a few case series and case reports are available that particularly describe rhinological, otological or other manifestations of EGPA in the ENT-region. Therefore, the objective of this study is to systematically describe data on ENT-region involvement in a large series of EGPA patients. METHOD: EGPA patients examined in the Department of Otorhinolaryngology of the Christian-Albrechts-University of Kiel between 1990 and 2010 were included in the study. Criteria for ENT-manifestation were assigned to five subgroups (history, ENT examination, audiological and rhinological diagnostic findings and cranial MRI) and documented cumulatively. EGPA patients were examined in a standardized way based on the validated Ear Nose and Throat Activity Score (ENTAS) or its precursor, including audiological and rhinological diagnostic findings. MRI scans were analysed to further evaluate ENT involvement. RESULTS: A total of 95 EGPA patients were included in the study. In approximately 80% of them, ENT-involvement was documented and the assumption of a frequent rhinological manifestation in patients with EGPA was confirmed. Moreover, the data reveals remarkable evidence for an otological manifestation. A missing correlation between the rhinological and the otological manifestation indicates an independent autoimmune-inflammatory process for this manifestation. CONCLUSION: The data of the largest monocentric study presented here confirms the hypothesis of a frequent ENT involvement in EGPA patients, in whom rhinological and otological manifestations are most common. Therefore, treatment should include long term follow-up and should be managed interdisciplinary.