Seccia Veronica, Baldini Chiara, Latorre Manuela, Gelardi Matteo, Dallan Iacopo, Cristofani-Mencacci Lodovica, Sellari-Franceschini Stefano, Bartoli Maria Laura, Bacci Elena, Paggiaro Pierluigi
1st Otorhinolaryngology Unit, Department of Neuroscience, Azienda Ospedaliero-Universitaria Pisana, Pisa, Italy.
Rheumatology Unit, University of Pisa, Pisa, Italy.
Int Arch Allergy Immunol. 2018;175(1-2):61-69. doi: 10.1159/000484602. Epub 2018 Jan 23.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a necrotizing vasculitis that predominantly affects small- to medium-sized vessels. It is characterized by a wide spectrum of extrapulmonary symptoms, including sinonasal and paranasal sinus abnormalities. These are the most common features of this disease, constituting diagnostic criteria for EGPA. However, the actual clinical features, cellular mechanisms and impact on patients' quality of life (QoL) are still a matter of study.
Thirty-nine EGPA patients underwent multidimensional rhinological evaluations, including rhinofibroscopy, nasal cytology, and QoL questionnaires. This was coupled with respiratory and rheumatological assessments.
Twenty-eight patients were diagnosed with chronic rhinosinusitis (CRS). Of these, 18 had nasal polyposis (NP). Chronic rhinitis was diagnosed in 10 patients. Of these, 3 had allergic rhinitis (AR) and seven had non-AR (NAR). Overall, only 1 patient (2.6%) was normal. Nasal cytology showed that hypereosinophilia was present in 17/28 patients with CRS, 4/7 patients with NAR and all patients with AR. SNOT-22 and SF-36 showed a severe impact of nasal symptoms on QoL. No differences in asthma control or rheumatological patterns for EGPA were observed among patients with or without NP.
Even when the rheumatological assessment scored EGPA "under control" according to the Birmingham Vasculitis Activity Score and Vasculitis Damage Index, sinonasal diseases and related nasal inflammatory processes were not controlled. Therefore, there is a need for clinical monitoring and targeted treatment to control the inflammatory processes and improve the QoL of EGPA patients.
嗜酸性肉芽肿性多血管炎(EGPA)是一种主要累及中小血管的坏死性血管炎。其特征为广泛的肺外症状,包括鼻窦和鼻旁窦异常。这些是该疾病最常见的特征,构成了EGPA的诊断标准。然而,其实际临床特征、细胞机制以及对患者生活质量(QoL)的影响仍有待研究。
39例EGPA患者接受了多维鼻科评估,包括鼻纤维镜检查、鼻细胞学检查和生活质量问卷。同时进行了呼吸和风湿病学评估。
28例患者被诊断为慢性鼻窦炎(CRS)。其中,18例有鼻息肉(NP)。10例患者被诊断为慢性鼻炎。其中,3例有过敏性鼻炎(AR),7例有非过敏性鼻炎(NAR)。总体而言,只有1例患者(2.6%)正常。鼻细胞学检查显示,17/28例CRS患者、4/7例NAR患者和所有AR患者均存在嗜酸性粒细胞增多。SNOT-22和SF-36显示鼻症状对生活质量有严重影响。有无NP的EGPA患者在哮喘控制或风湿病模式方面未观察到差异。
即使根据伯明翰血管炎活动评分和血管炎损伤指数,风湿病学评估显示EGPA“得到控制”,鼻窦疾病和相关的鼻炎症过程仍未得到控制。因此,需要进行临床监测和针对性治疗,以控制炎症过程并改善EGPA患者的生活质量。