Focus on the Involvement of the Nose and Paranasal Sinuses in Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): Nasal Cytology Reveals Infiltration of Eosinophils as a Very Common Feature

Abstract

Background: 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. Methods: Thirty-nine EGPA patients underwent multidimensional rhinological evaluations, including rhinofibroscopy, nasal cytology, and QoL questionnaires. This was coupled with respiratory and rheumatological assessments. Results: 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. Conclusions: 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.

European position paper on diagnostic tools in rhinology

“…Eosinophilic granulomatosis with polyangiitis (EGPA) is a necrotizing vasculitis of small and medium-sized vessels. Among a wide spectrum of non-pulmonary symptoms, nasal and sinonasal features are common and part of the diagnostic criteria for EGPA (62) . (63) .…”

Upper respiratory tract involvement in patients with ANCA-associated vasculitis

“…При проведении цитологического исследования мазка со слизистой оболочки носа у больных ЭГПА с симптомами хронического риносинусита V. Seccia и соавт. обнаружили гиперэозинофилию в 17 из 28 случаев [47]. При гистологическом исследовании обычно выявляют некротическое гранулематозное воспаление в сочетании с тканевой эозинофилией [1,48,49].…”

“…Ранее сходные данные были получены V. Seccia и соавт. [47]. По мнению авторов, полученные результаты могут указывать на развитие независимого аутоиммунного воспалительного процесса в области ВДП и органа слуха, подобного локальным формам ГПА, что обосновывает необходимость долгосрочного наблюдения и междисциплинарного подхода к лечению.…”

Chronic rhinosinusitis in eosinophilic granulomatosis with polyangiitis: clinical presentation and antineutrophil cytoplasmic antibodies

“…Although asthma and eosinophilia are hallmark traits of this disease, patients also commonly present with pulmonary infiltrates or eosinophilic pneumonia, peripheral neuropathy, skin involvement, and rhinologic disease . Sinonasal cavity involvement, including rhinitis, polyposis, and sinusitis, occurs in roughly 50% of EGPA patients with estimated prevalence of chronic rhinosinusitis (CRS) being between 14.2% and 72% . Existing studies describing the EGPA manifestations in the sinonasal cavity have thus far only broadly described the sinonasal disease in EGPA patients by identifying general sinonasal involvement .…”

“…2 Sinonasal cavity involvement, including rhinitis, polyposis, and sinusitis, occurs in roughly 50% 2, 6 of EGPA patients with estimated prevalence of chronic rhinosinusitis (CRS) being between 14.2% and 72%. [6][7][8] Existing studies describing the EGPA manifestations in the sinonasal cavity have thus far only broadly described the sinonasal disease in EGPA patients by identifying general sinonasal involvement. 6,7,[9][10][11] To our knowledge, there have been no studies to comprehensively describe the presentation patterns and outcomes after management of CRS in EGPA.…”