Andersen Henrik, Götz Paul, Bremer Jan Phillip, Laudien Martin
Klinik für HNO-Heilkunde, Kopf- und Halschirurgie, Christian-Albrechts-Universität zu Kiel, Arnold-Heller-Str. 3, Haus 27, 24105, Kiel, Deutschland.
Klinik für Rheumatologie und Immunologie, Klinikum Bad Bramstedt, Bad Bramstedt, Deutschland.
Z Rheumatol. 2018 Dec;77(10):928-937. doi: 10.1007/s00393-018-0439-0.
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.
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 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 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.
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.
嗜酸性肉芽肿性多血管炎（EGPA）是一种病因不明的罕见的中小血管肉芽肿性血管炎。已知头颈部区域常受累。由于尚不清楚头颈部区域的炎症过程是否与全身疾病活动平行，因此在一项对比研究中对全身和局部疾病活动的进程进行了调查。
记录了1990年至2010年间25例患者的疾病进展情况，共进行了90次会诊，平均观察期为7.02年。进行了病史采集、临床观察和检查以描述耳鼻喉（ENT）表现。此外，还收集了全身数据，如伯明翰血管炎活动评分（BVAS）、血管炎损伤指数（VDI）以及血清学参数C反应蛋白（CRP）、嗜酸性阳离子蛋白（ECP）和嗜酸性粒细胞绝对计数（EOS）。为了确定头颈部区域的活动情况，对现有的表现进行了总结，并使用Spearman等级相关系数与全身数据进行统计学比较。
总体而言，统计分析显示耳鼻喉区域的疾病活动与全身疾病活动之间的相关性非常弱，不认为具有显著性。将鼻部区域的活动与全身活动进行比较时，在所有会诊中有22.9%发现了显著值。将耳部区域的活动与全身活动进行比较时，所有会诊中有11.8%显示出显著值。
结果表明头颈部区域的疾病活动与全身活动无关。此外，它们指出了上述区域潜在的独立自身免疫性炎症过程；因此，治疗应包括长期随访和多学科管理。
